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1st CONGRESS of the EUROPEAN YOUNG PAEDIATRICIANS
YOU N TPA 1930 NS IA G DI PAE ATRIC 1st CONGRESS of the EUROPEAN YOUNG PAEDIATRICIANS’ ASSOCIATION Abstract Book & Congress Information Wyndham Grand Levent Hotel 4 - 6 December 2015 Istanbul/TURKEY CONTENTS WELCOME.................................................................................2 COMMITTEES............................................................................3 SPEAKERS AND MODERATORS ....................................................4 LOCAL INFORMATION..............................................................5-6 USEFUL TURKISH EXPRESSIONS..................................................7 CONGRESS INFORMATION..........................................................8 SOCIAL PROGRAM.....................................................................8 VENUE PLAN.............................................................................9 OUR SUPPORTERS................................................................... 10 SCIENTIFIC PROGRAM......................................................... 11-16 ABSTRACTS .................................................................... 17-128 INDEX.................................................................................. 129 -1- Dear Friends and Colleagues, EURYPA (European Young Paediatricians’ Association) is founded in January 2015 primarily by the Turkish Young Paediatricians and ONSP (Italian National Observatory for Paediatric Residents). Our main aim is to facilitate networking among paediatricians all over Europe and support them on their quest for better education during and post-specialization, research opportunities. We’re delighted to welcome you to the 1st ever Congress of EURYPA where newly specialized paediatricians and paediatric residents will meet, present their interesting cases with an emphasis on the main topic supported by their senior doctors. There will be workshops about the problems we’re facing and the future projects to tackle them as well. No need to say, there will also be a rich social programme for us to get to know each other in the heart of Istanbul. EURYPA 1st Congress Organizing Committee -2- EURYPA 1st CONGRESS ORGANIZING COMMITTEE EPA-UNEPSA COUNCIL SERHAT GÜLER DAVIDE VECCHIO MİNE ÖZDİL SALVATORE AVERSA ADEM KARBUZ PINAR ÜRENDEN ELİÇİN PAOLA BERLESE ERTUĞRUL KIYKIM ROBERTO RASCHETTI LEYLA NAMAZOVA-BARANOVA President MASSIMO PETTOELLO-MANTOVANI Secretary General JULIJE MESTROVİC Vice President MANUEL MOYA Vice President GİZEM PAMUK ZEYNEP İŞÇAN TUĞÇE KALAYCI ORAL ÇİĞDEM ORUÇ AYŞE AYZIT KILIÇ SERAP SAPMAZ SEVİNÇ GÜMÜŞ ZEYNEP ALP ÜNKAR ÜMMÜHAN ÖNCÜL BURÇİN NAZLI KARACABEY TUBA KOÇKAR ELİF ARSLAN GULNARA HEYDAROVA ADA BULUT DORUK GÜL ZEYNEP KARACA PINAR YAMAÇ ENSAR DURAS MELDA TAŞ NAZLI KAVCIK AYŞE PEHLEVAN HATİCE EREN MUSTAFA ÖZDEMİR ERKAN ERFİDAN REFİA GÖZDENUR SAVCI FÜGEN ÇULLU ÇOKUĞRAŞ Councilor SIMON LENTON Councilor JOCHEN EHRICH Treasurer ANDREAS KONSTANTOPOULOS Past President MARIN BURLEA President of 8th Europaediatrics MEHMET VURAL President of Balkan Pediatrics Association Secretary of TPA GENERAL SECRETARY OF EURYPA 1st CONGRESS ÖMER FARUK BEŞER -3- SPEAKERS AND MODERATORS SPEAKERS AND MODERATORS FROM TURKEY Ahmet Arvas Ada Bulut Sinoplu Adem Karbuz Aysun Karabay Bayazıt Ayşe Ayzıt Atabek Ayşe Güler Eroğlu Beyhan Tüysüz Burak Doğangün Burçin Nazlı Karacabey Bülent Hacıhamdioğlu Çiğdem Oruç Diğdem Bezen Elif Arslan Emel Gür Emin Ünüvar Emine Olcay Yasa Engin Tutar Ergin Çiftçi Ertuğrul Kıykım Esra Özek Fatih Varol Fatma İrioğlu Kocael Fuat Gürkan Gizem Pamuk Gözde Apaydın Gözde Yücel Gülnara Heydarova Hacer Ergin Haluk Çokuğraş Hasret Ayyıldız Civan Hatice Öztürk Hilmi Apak Hüray Kök Hüseyin Kılıç Kenan Barut Levent Saltık M.Tayyib Kadak Mehmet Satar Mehmet Vural Melike İrem Petan Merve Erdemir Kula Mine Özdil Mustafa Akçam Mustafa Kemal Özdemir Müjgan Alikaşifoğlu Münevver Kaynak Türkmen Nafiye Urgancı Nazlı Kavçık Nihat Sapan Nilay Güneş Olcay Evliyaoğlu Oya Ercan Ozan Özkaya Ömer Faruk Beşer Övgü Kul Çınar Özden Aksu Sayman Özgür Kasapçopur Pınar Yamaç Dilaver Raşit Vural Yağcı Refia Gözdenur Savcı Salim Çalışkan Sebuh Kuruğoğlu Sema Saltık Serhat Güler Sezgin Şahin Sultan Kavuncuoğlu Süheyla Ocak Şükrü Çekiç Tiraje Celkan Tuba Koçkar Tuğba Erener Ercan Tülay Erkan Ümmühan Öncül Vildan Ertekin Yıldız Perk Yücel Taştan Zeynep İşçan Zeynep Tamay INTERNATIONAL SPEAKERS AND MODERATORS Alma Topi Ana Skelin Ana Stefanescu Ancuta Ignat Angelica Cristina Marin Bendavid Matthieu Davide Vecchio Evda Vevecka Jelena Sabljic Julia Jillich Julije Mestrovic Kerbl Reinhold Manuel Ferreira-Magalhaes Margareta Seher Zupancic Marin Burlea Massimo Pettoello Mantovani Norbert Varga Paola Berlese -4- Rita Lónyai Roberto Raschetti Sara Gorgoglione Sebastian Grey Sharat Varma Tatjana Ardalic Tudor L Pop Ulrike Wanz Vasile Valeriu Lupu Zsuzsanna Almassy LOCAL INFORMATION Hotel Name Wyndham Grand Levent Hotel Hotel Address Esentepe Mah. Büyükdere Cad. No:177-183 34394 Şişli – İstanbul Getting Around Istanbul has an extensive public transport network with its highway, rail and funicular systems and sea transport services. The easiest way to reach the Congress Venue, is the take the metro line and use station “ Levent”. -5- Transportation Cards The city has an integrated electronic ticket system which uses smart RFID cards called İstanbulKart. It’s a contactless smart card used at public transport in the city and can be acquired at major transport interchanges. The main benefit of İstanbulKart is to save time and money while travelling. The easy to use and easy to obtain reasonably priced İstanbulKart may be used in any public transportation throughout the city eith the benefit of cheaper rates compared to buying a ticket or token. Also, the card can be loaded at special machines or some kiosques which can be found almost in every main bus stations and ferry stations. Weather in Istanbul Current forecast show warmer than usual temperature, however be ready for cold weather and occasional rain during congress dates. Banking and Exchange Facilities Currency is the Turkish Lira. Foreign money can be changed by banks during working days (09.00- 17.00 Monday Friday) as well as hotels, exchange offices. Exchange rates are set daily by the Central Bank. All major credit cards are accepted in most of the hotels, restaurants and shops. Automated bank machines are available at many points throughout the city and the airport. Shopping One of the most enjoyable parts of a trip to Turkey is shopping for the rich variety of Turkish crafts. Telephones Pay phones and credit card phones are available at the meeting venue as well as in the city. Electricity The electrical power supply in Turkey is 220 volts. Tipping Although service charge is included in most restaurants, leaving a tip at one’s own discretion is appreciated. -6- USEFUL TURKISH EXPRESSIONS English Turkish Pronunciation Hello Merhaba! MARE-HA-BA see above Good evening İyi akşamlar! EE AK-SHAM-LAR Hoşçakal! HOSH-CHA-KAL Güle güle! GUU-LEH GUU-LEH How are you? Nasılsın? NA-SEL-SIN I am fine, and you? İyiyim, sen nasılsın? EE-IM, SEN NA-SEL-SIN Thank you Teşekkür ederim. TE-SH-QU-ERR ED-ERR-IM Good-bye (said by person leaving) Good-bye (said by person staying) You’re welcome Birşey değil. (in response to “Thank you”) BEER-SHEY DEY-EEL Please Lütfen. LUT-FEN I don’t speak Turkish Türkçe bilmiyorum! TURK-JEH BILL-MI-YOR-UM I don’t understand Sizi anlamıyorum. SI-ZI ANN-LA-MA-YOR-UM Could you please repeat Şunu tekrar edebilir misiniz? SHU-NU TEK-RA ED-EBB-ILL-EAR MISS-IN-IZ Tanıştığımıza sevindim./Memnum TAN-ISH-TI-IM-IZA SEV-IN-DIM/ MEM-NUM- oldum OLL-DUM How much does it cost? Ne kadar? NAY KAD-AR I am sorry Özür dilerim. OUZ-UR DILL-EAR-IM Excuse me Afedersiniz. AFF-ED-DAR-SAN-IZ Can you help me? Yardım edebilir misiniz? YARR-DIM ED-EBB-ILL-EAR MISS-IN-IZ Yes Evet. EV-ET No Hayır. HI-EAR I don’t know Bilmiyorum. BILL-ME-YOR-UM Okay/Alright Tamam. TAM-AM Welcome Hoş Geldiniz. HOSH GEL-DIN-IZ Pleased to meet you -7- CONGRESS INFORMATION Certification of Attendance All congress participants will be issued a certification of attendance. You can collect your certificates from the registration desk from December 06th, 2015. Name Badge All participants and accompanying people are kindly advised to wear their name badges at all times during the congress. If you lose your name badge, please apply to registration desk. Poster Exhibition Poster area is located in the Hezarfen Ahmet Celebi Hall. All poster presenters are expected to be with their posters 6 December 2015, between 18.00-19.00 for poster evaluation. Congress Registration Desk Congress registration desk will be at the entrance of the congress center and will be open from 07:00 to 19:00 during congress dates for giving information about registration and accommodation. SOCIAL PROGRAM 4 December 2015 – Wyndham Grand Hotel 18.00-22.00 Opening Ceremony - Violin and Piano Performance, by Müge Sak and Zümrüt Alieva - Opening Remarks - Jazz Concert and Dance Party- Ezgi Ayça Kızıldere 5 December 2015 – Wyndham Grand Hotel 19.00-22.00 Gala Dinner - Concert: Demet Tuncer : Demet Tuncer started her musical career in Las Vegas and Santa Fe with Austin Miskel and Andre Garcia Nuthman. Demet also had the privilege of working with the current Dean of Drama and Dance at the University of Houston, Steve Wallace, who also pioneered an academic play ‘Prymates’, in Broadway. -8- -9- WE GRATEFULLY THANK TO OUR SPONSORS FOR THEIR VALUABLE SUPPORT FOR THE 1ST CONGRESS OF EURYPA - 10 - 04 Aralık 2015, Cuma SAAT PEDİATRİK RADYOLOJİ KURSU ÇOCUK KARDİYOLOJİ KURSU BESLENME KURSU SAĞLAM ÇOCUK İZLEMİ KURSU YENİDOĞAN KURSU 09.00 10.00 Pediatrik Toraks Radyolojisi Sebuh Kuruğoğlu Üfürümü Olan Çocuğa Yaklaşım Ayşe Güler Eroğlu Çocukluk Çağında Besin İhtiyaçları ve Malnütrisyonun Tanınması Engin Tutar Sağlam Çocuk İzlemi Emel Gür Olgularla Yenidoğanda Mekanik Ventilasyon Mehmet Vural Çocukluk Çağında Aşılanma Ahmet Arvas Sarılığa Güncel Yaklaşım Yıldız Perk Adölesan İzlemi Müjgan Alikaşifoğlu Prematüre Bebeğin Parenteral-Enteral Beslenmesi Sultan Kavuncuoğlu 10:00 10:15 10:15 11:15 KAHVE ARASI Pediatrik Nöroradyoloji Sebuh Kuruğoğlu Telekardiyografinin Değerlendirmesi Ayşe Güler Eroğlu 11:15 11:30 11:30 12:30 KAHVE ARASI Pediatrik Batın Radyolojisi Sebuh Kuruğoğlu Elektrokardiyografinin Değerlendirmesi Ayşe Güler Eroğlu 12.30 13.30 13.30 15.00 Malnütrisyon Tedavisinde Enteral Beslenme Vildan Ertekin Malnütrisyon Tedavisinde Parenteral Beslenme Nafiye Urgancı ÖĞLE YEMEĞİ 13.30-14.15 Kistik Fibroz; Beslenmede Güncel Yaklaşımlar ve Öneriler Fügen Çullu Çokuğraş Gülnara Heydarova Ömer Faruk Beşer 14.15-15.00 Kistik Fibroz; Akciğer Tutulumunda Koruyucu Önlemler ve Tedavide Güncel Yaklaşımlar Haluk Çokuğraş Ayşe Ayzıt Atabek Tuba Koçkar 15.00 15.30 KAHVE ARASI UYDU SEMPOZYUMU 15.30 16.30 16.30 18.00 18.00 19.00 19.00 21.30 Erken Dönem Beslenmenin Önemi ve Flora Gelişimine Etkisi Mehmet Vural Raşit Vural Yağcı 16.30-17.15 Demir Eksikliği Anemisi Hilmi Apak Nazlı Kavçık Hüray Kök 17.15-18.00 Lenfadenopatili Çocuğa Yaklaşım Tiraje Celkan Süheyla Ocak Gözde Yücel AÇILIŞ TÖRENİ ve MÜZİK DİNLETİSİ (Müge Sak, Zümrüt Alieva) JAZZ KONSERİ ve DANS PARTİSİ (Ezgi Ayça Kızıldere) - 11 - 05 Aralık 2015, Cumartesi SAAT PROF. DR. SEZAİ BEDRETTİN TÜMAY SALONU PROF. DR. ŞÜKRAN YALÇINDAĞ SALONU 09.00 10.30 09.00-09.45 Çocuklarda Boy Kısalıkları Oya Ercan Ada Bulut Sinoplu Övgü Kul Çınar 09.45-10.30 Erken Püberte Olcay Evliyaoğlu Diğdem Bezen Hatice Öztürk 09.00-09.45 Ateşli Hastaya Yaklaşım Yücel Taştan Çiğdem Oruç Fatih Varol 09.45-10.30 Çocuklarda Antibiyotik Kullanımında Doğrular-Yanlışlar - AKILCI İLAÇ KULLANIMI OTURUMU Emin Ünüvar Adem Karbuz Ümmühan Öncül 10.30 11.00 11.00 12.00 KAHVE ARASI Aşılar Hakkında Kanıta Dayalı Bilimsel Yaklaşımlar Ahmet Arvas Aşı takviminde uygulanan aşılar Gizem Pamuk Yeni aşılar Gözde Apaydın UYDU SEMPOZYUMU (Besin Alerjileri; Güncel Tedavi Yaklaşımları) Haluk Çokuğraş Ömer Faruk Beşer 12.00 13.00 13.0014.00 14.00 15.00 15.00 16.00 16.00 16.30 Hangi Çocukta Metabolik Hastalık Düşünelim? Ertuğrul Kıykım Melike İrem Petan Refia Gözdenur Savcı Özden Aksu Sayman UYDU SEMPOZYUMU (Nörolojik Hastalarda Malnütrisyon ve Tedavi Yaklaşımları) Tülay Erkan Serhat Güler Yenidoğan Solunum Problemlerine Güncel Yaklaşımlar Mehmet Satar Mine Özdil Zeynep İşçan Genetik; Depo hastalıklarında Güncel Tanı-Tedavi Yaklaşımları Beyhan Tüysüz Nilay Güneş Ertuğrul Kıykım KAHVE ARASI - 12 - 05 Aralık 2015, Cumartesi SAAT PROF. DR. SEZAİ BEDRETTİN TÜMAY SALONU PROF. DR. ŞÜKRAN YALÇINDAĞ SALONU 16.30 17.30 Probiyotiklerin hayatımızda ve güncel tıptaki yeri Raşit Vural Yağcı Akut ishalde Probiyotikler Adem Karbuz Alerjik hastalıklarda Probiyotikler Pınar Yamaç Dilaver Fonksiyonel Sindirim Sistemi Hastalıklarında Probiyotikler Merve Erdemir Kula Enflamatuar Barsak Hastalıklarında Probiyotikler Hasret Ayyıldız Civan Febril Konvülsiyon: Yaklaşım Ve Tedavide Yenilikler Sema Saltık Hüseyin Kılıç Burçin Nazlı Karacabey Alerjik Cilt Hastalıkları Zeynep Tamay Esra Özek 17.30 18.00 18.00 19.00 POSTER 20.00 22.00 GALA YEMEĞİ KONSER: DEMET TUNCER 06 Aralık 2015, Pazar SAAT PROF. DR. SEZAİ BEDRETTİN TÜMAY SALONU 09.00 10.30 09.00-09.45 Çocuk Psikiatrisinde Gelişimsel Bozukluklar Burak Doğangün M.Tayyib Kadak 09.45-10.30 Solunum yolu alerjileri; Güncel Tedavi ve Önlemler Nihat Sapan Fatma İrioğlu Kocael Şükrü Çekiç 10.30 11.00 11.00 12.30 KAHVE ARASI 11.00-11.45 Çocuklarda Otoenflamatuar Hastalıklar Özgür Kasapçopur Sezgin Şahin Kenan Barut 11.45-12.30 Tekrarlayan İdrar Yolu Enfeksiyonlarında Güncel Yaklaşımlar Salim Çalışkan Elif Arslan Mustafa Kemal Özdemir 12.30 13.30 ÖĞLE YEMEĞİ 13.30 KAPANIŞ - 13 - 04 December 2015, Friday TIME PROF. DR. ÖZDEMİR İLTER HALL 09.00 10.30 COURSE 10.30 11.00 COFFEE BREAK 11.00 12.30 COURSE 12.30 13.30 LUNCH 14.15 15.00 “Aspects of Gastroesophageal Pathology in Children” Moderator: Marin Burlea • Particularities of the Gastroesophageal Reflux Disease in Childhood” Vasile Valeriu Lupu • Gastritis in Childhood” Ancuta Ignat • Helicobacter Pylori Infection in Children” Angelica Cristina Marin 15.00 15.30 COFFEE BREAK SATELLITE SYMPOSIUM * 15.30 16.30 16.30 17.30 Importance of Early Life Nutrition and Effect on Development of Microbiota Mehmet Vural Raşit Vural Yağcı “Paediatric/neonatal emergencies” Moderator: Julije Mestrovic • “Coma, case presentation” Tatjana Ardalic • “Apnoea, case presentation” Ana Skelin • “Dyspnoea, case presentation” Jelena Sabljic 18.0019.00 OPENING CEREMONY and CONCERT (Müge Sak, Zümrüt Alieva) 19.0021.30 JAZZ CONCERT and DANCE PARTY (Ezgi Ayça Kızıldere) *The symposium will be Turkish, but simultaneous translation will be available in English. - 14 - 05 December 2015, Saturday TIME 09.00 10.30 PROF. DR. ÖZDEMİR İLTER HALL 09.00-09.45 “Sudden Infant Death Syndrome” Moderator: Kerbl Reinhold • “Epidemiology and pathogenesis” Julia Jillich • “Risk factors and prevention” Ulrike Wanz 09.45-10.30 “EURYPA Executive Commitee and Board Meeting” Moderator: Pınar Ürenden Eliçin Davide Vecchio Mine Özdil Sebastian Grey 10.30 11.00 11.00 12.00 12.00 13.00 COFFEE BREAK EURYPA Executive Commitee and Board Meeting Moderator: Pınar Ürenden Eliçin Davide Vecchio Mine Özdil Sebastian Grey SATELLITE SYMPOSIUM * Food allergies; Current Treatments Haluk Çokuğraş Ömer Faruk Beşer 13.00 14.00 14.00 15.00 15.00 16.00 LUNCH SATELLITE SYMPOSIUM * Malnutrition in neurological diseases Tülay Erkan Serhat Güler “Ins and Outs of the New Neonatal Resuscitation Guidelines” Moderators: Massimo Pettoello Mantovani and Mehmet Vural • “Interactive and multimedial case reports” Davide Vecchio Paola Berlese Roberto Raschetti • Lecturer: Davide Vecchio • Commentator: Mehmet Vural 16.00 16.30 16.30 17.30 COFFEE BREAK “Career Planning for Young Pediatricians” Moderator: Massimo Pettoello-Montovani (Round table session) 17.30 18.30 POSTER SESSION 20.00 22.00 GALA DINNER-CONCERT: DEMET TUNCER *The symposium will be Turkish, but simultaneous translation will be available in English. - 15 - 06 December 2015, Sunday TIME 09.00 10.30 PROF. DR. ÖZDEMİR İLTER HALL 09.00-09.45 “Supplementation of vitamins and micronutrients in infants” Moderator: Fügen Çullu Çokuğraş • “D vitamin supplementation and prophylaxis” Zeynep Hızlı • “Iron supplementation and prophylaxis” Erkan Erfidan • “Supplementation of the other micronutrients” Zeynep Alp Ünkar 09.45-10.30 “Pediatric hepatology” Moderator: Tudor Pop • “Non-invasive evaluation of the fibrosis in children” Ana Stefanescu • “Indications of liver transplantation in children” Sharat Varma 10.30 11.00 11.00 12.30 COFFEE BREAK 11.00-11.45 ‘’Metabolic diseases in children’’ Moderator: Zsuzsanna Almassy • “’A Rare Cause Behind Developmental Regression in Infancy” Rita Lónyai • “’The Presence of Hunter Sydrome in a Family” Norbert Varga 11.45-12.30 “Acute respiratory disease in infancy; last evidence” Moderator: Ergin Çiftçi • Bordetella pertussis infection in a 1 month old infant, with respiratory failure Manuel Ferreira-Magalhaes • High flow nasal canula treatment in bronchiolitis Sebastian Gray 12.30 13.30 LUNCH 13.30 CLOSING CEREMONY - 16 - ABSTRACTS - 17 - [P-001] [P-002] G. V. Volynets, T. A. Skvortsova, E. L. Semikina, A. N. Surkov, A. V. Nikitin, A. O. Anushenko Gastroenterology and Hepatology Department, Scientific Centre of Children Health of the Ministry of Health of the Russian Federation, Moscow, Russian Federation Background&Aims: The aim of the study was to determine the predictors of sustained virologic response (SVR) to interferon alpha-2b plus ribavirin therapy in chronic hepatitis C children. Fatih Çelmeli1, Abdullah Kocabaş2, A.ishak Işık3, Mesut Parlak4, Serdar Ceylaner5 1 Department of Pediatric Allergy-Immunology, Antalya Education and Research Hospital, Antalya, Turkey 2 Department of Pediatric Cardiology, Antalya Education and Research Hospital, Antalya, Turkey 3 Department of Pediatric Gastroenterology, Antalya Education and Research Hospital, Antalya, Turkey 4 Department of Pediatric Endocrinology, Antalya Education and Research Hospital, Antalya, Turkey 5 İntergen Genetic Diseases Diagnostic Center, Ankara, Turkey A study of best treatment predictors of sustained virologic response to interferon alpha-2b plus ribavirin therapy in children with chronic hepatitis C Methods: 29 children and adolescents (mean age 9.1±0.8 years) were chronically infected with HCV genotype 1 (58.6%) and HCV genotype 2 or 3 (41.4%). All the patients were treated with PEG-IFN alfa-2b (60 microg/m(2)/week) plus RBV (15 mg/kg/day). The prediction performance was assessed using receiver operating characteristics (ROC), correlation analyse of various clinical, laboratory and anthropometric parameters. Results: The following pre-treatment predictors of SVR showed a good correlation: age (r=0.429, p=0.02), weight (r=0.85, p=0.000) and stature (r=0.966, p=0.000). Children with weight >23 kg (AUC=0.834), older than 6 yo (AUC=0.808), higher than 115 cm (AUC=0.828) were more likely to attain SVR. Other predictors of SVR were absolute lymphocyte count >2.5*109/L (R=0.506, p=0.05), absolute neutrophil count 2-3.4*109/L (R=0.561, p=0.002) and serum interferon level 16-22 U/ml (R=0.669, p=0.000). Conclusions: The identified factors significantly predict SVR to PEG-INF and ribavirin on pretreatment stage. Success rate of treatment based on these predictors reaches up to 83% regardless of HCV genotype. Keywords: HCV, predictors, SVR Unexplained cyanosis revealing hepatopulmonary syndrome in a girl with APECED syndrome caused by a novel AIRE mutation Background&Aims: Autoimmune polyendocrinopathy type 1, also called autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) is a rare primary immunodeficiency disease caused by lossof-functions mutations in the AutoImmune REgulator (AIRE) gene on chromosome 21q22.3. AIRE gene is an important mediator of tolerance to self-antigens. The clinical spectrum of the disease is characterized by a wide heterogeneity because of the highly variable pattern of destructive autoimmune reactions toward different endocrine and non-endocrine organs. Results: A 17-year-old girl was referred to our hospital with complaints of cyanosis and dyspnea. Peripheral oxygen saturation was 73%. Also, she had diffuse oropharyngeal moniliasis, skin hyperpigmentation, central cyanosis and digital clubbing. She was following by pediatric endocrinology department with the diagnoses of Addison’s disease, hypoparathyroidism, and hyper gonadotropic hypogonadism. She had recurrent chronic mucocutaneous candidiasis since 6 years of age and pangastritis. Her clinical manifestations were consistent with APECED, which was later confirmed by the identification of a novel homozygous mutation p.R15H (c.44G>A) in the AIRE gene. The blood gase assessment revealed PaO2: 55.8 mmHg, PaCO2: 21.7 mmHg, Sat O2: 88 %. CT revealed nodular contour and heterogeneity of the liver. The patient experienced upper gastrointestinal bleeding, and esophagoduedenoscopy showed esophageal varices and candiasis. The portal venous Doppler showed a dilated portal vein with a large thrombus. Liver biopsy revealed autoimmune hepatitis which is a rare manifestation of APECED Syndrome. She had prominent platypnea and orthodeoxia. The contrast-enhanced echocardiography with agitated saline was compatible with an intrapulmonary right-to-left shunt after 4th cardiac cycle, thus the diagnosis hepatopulmonary syndrome (HPS) was confirmed in the patient. Conclusions: Herein, we reported a girl with a novel mutation in the AIRE gene, and complicated by HPS due to autoimmune hepatitis and portal hypertension. To the best of our knowledge, HPS has not been reported as a manifestation of APECED Syndrome previously. Keywords: APACED, hepatopulmonary syndrome, novel mutation - 18 - [P-003] present this in the following case study. Articular Involvement in Childhood Familial Mediterranean Fever Kenan Barut1, Ada Bulut Sinoplu2, Gözde Yücel2, Gizem Pamuk2, Amra Adrovic1, Sezgin Şahin1, Özgür Kasapçopur1 1 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatric Rheumatology 2 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatrics Introduction: FMF is an autosomal recessively inherited autoinflammatory disease which is clinically manifested with periodic episodes of fever, serositis and arthritis. Articular involvement is also a frequent presentation after fever and peritonitis in childhood FMF. Objective: The aim of this study is to evaluate the demographic and clinical features of the common articular findings in the course of FMF and compare these with the relatively rare occasions of chronic arthritis and FMF comorbidity. Patients and Results: Among the 708 patients diagnosed with FMF; arthritis was defined in 288 (40,7%) cases. In 192 (66%) children the affected joints were observed primarily as the ankles, subsequently followed by knees in 148 (51%) cases. Articular findings were mainly acute monoarthritis without leading to sequelae. When the children presented with arthritis were considered, the mean age at disease onset was estimated to be 5,2 ± 3,7 years and the mean age at diagnosis was 8,1±4 years. The mean duration of the episodes of FMF mainly manifested by articular symptoms was yielded as 97 ± 53,6 hours (with the median of 72 hours,ranging between 24-168 hours) and when compared with the cases primarily manifested with abdominal pain and fever that was declared to be 59,4±34,3 hours in a former study. In 23 (8%) cases with the diagnosis of FMF and articular manifestations,a coincidental diagnosis of chronic arthritis was considered to be more consistent as the disease progressed. Conclusion: Articular involvement in FMF is acute monoarthritis typically affecting the lower extremities in a periodic manner with short duration and without sequelae formation. The clinical entity of chronic arthritis and FMF coincidence can emerge as a rare occasion. Especially with the cases of oligoarticular JIA that show recurrence and quickly respond to treatment, the diagnosis of FMF should also be kept in mind. Keywords: arthritis, familial mediterranean fever [P-004] Case report of a dystrophic child Ádám Schmidt, Margit Lörincz Heim Pál Children’s Hospital, Gastroenterology Department, Budapest, Hungary Background: It seems so, that the differential diagnosis of gluten-sensitive enteropathy can be still difficult. The illness can be so progressive that it can be close to coeliac crisis at the time it is diagnosed. I would like Case: A 20,5 month-old fatigue child was brought to the hospital’s Emergency Unit with the following symptoms: chronic diarrhoea, meteoristic belly, sleepiness. His GP was following the child’s case since he was 6 month old because of dystrophy, but there was no previous examination. Suspicion of Malabsorption syndrome indicated hospitalisation. Examinations shown hypoproteinaemia, hypalbuminaemia, iron deficiency anaemia. Endoscopy was indicated. Results: The critical symptoms were explained when the child was diagnosed with severe coeliac disease. It is still not clear what caused the flattening of his weight percentile before the exposition of gluten and what were the causes of the late diagnoses of this rather frequent disease. The health of the child improved rapidly with the coeliac diet. The follow-up showed that beside the coeliac disease there was another malabsorption disease present in the child’s system, which we could not identify. Conclusions: It is necessary to be aware that coeliac disease is frequent and it must be diagnosed in time. There is also the possibility that it appears in conjunction with other illnesses. Keywords: Gastroenterology, Coeliac disease, Dystrophy [P-005] A Well Known, But Less Diagnosed Disease: Pharyngoconjuntival Fever Adem Karbuz, Mustafa Kemal Özdemir, Alper Kaçar, Vefik Arıca Okmeydanı Eğitim ve Araştırma Hastanesi, Çocuk Kliniği, İstanbul, Türkiye Introduction: Pharyngoconjuntival fever is a common syndrome, include benign follicular conjunctivitis, pharyngitis, fever and cervical adenitis caused by Adenovirus serotype 3 and 7. It typically involves bilateral eyes. This syndrome generally self-limited and seen in pediatric population sporadically. In our case report, we would like to remember that a child with pharyngitis, non-purulent conjunctivitis and continuous fever unresponsive to antibiotic therapy is not necessarily sign of a serious infection. Case: 2.5 years old boy apply to emergency service with high fever, redness in eyes and cough. Reddening of the eyes began 6 days ago in left eye. Family doctor prescribed an antibacterial eye drop, 2 days later symptoms were aggravated, eyes became red, and body temperature was raised. Patient applied to emergency service, doctor prescribed amoxicillin & clavulanic acid for upper respiratory tract infections. Patient was experienced fever within 2 days, then patient applied to emergency service secondarily. Parenteral cefuroxim treatment was begun, but fever continued. Patient was hospitalized because has got fever unresponsive to oral and parenteral antibiotic treatment, bilateral conjunctivitis for 4 days. In physical examination, he has bilateral serous conjunctivitis, oropharyngeal hyperemia, - 19 - tonsillar hypertrophy and multiple jugulodigastric and anterior cervical lymphadenopathies up to 1,5*1 cm in diameter. The other part of body is normal. Patient’s laboratory findings are; white blood cells: 8700/mm3, hemoglobin level: 10,9 g/dl, platelet count: 277000/ mm3, C-reactive protein: 70 mg/l. Other blood biochemical levels are normal. Adenovirus PCR test is positive in nasopharynxgeal aspiration specimen. We have started intavenous fluid treatment and stopped antibiotic treatment. We observed the patient, symptoms are relieved, was not experience fever and, conjunctivitis was healed after 2 days hospitalization. Keywords: Pharyngoconjunctival Fever, adenovirus [P-006] Premature Baby Vaccination; Could It Be Done On Time? Adil Umut Zübarioğlu, Mesut Dursun, Sinan Uslu, Ali Bülbül, Duygu Besnili Acar, Evrim Kıray Baş, Ebru Türkoğlu Ünal Department of Neonatology, Sisli Hamidiye Etfal Education and Research Hospital, Istanbul, Turkey Background&Aims: In this study the aim is to investigate vaccination status during hospitalization and factors that effects this situation in babies followed in outpatient clinics. Methods: The study is cross-sectional; all babies admitted in high risk newborn outpatient clinic between October 2013 to end of March 2014 and October 2014 to end of March 2015 (palivizumab prophylaxis session) included. Babies divided in 2 groups according to hospital that gave care. Group 1 was our hospital, Group 2 was external center. Demographic features, hospitalization time, gestational weeks, weight and vaccination status at discharge recorded. Results: One hundred thirty eight babies included in study whose all information could be taken. Sixty-seven babies (48.5 %) were in group 1. Mean gestational week was 27.4±1.8 weeks in group 1 and 28.5±1.9 weeks in group 2. Mean birthweight was 1085.2±268 grams in group 1 and 1216.8±415.7 grams in group 2. There were no statistical significance in demographic features between groups. First dose hepatitis B vaccine was delayed in 14 (10%) babies. From these babies 12 of them were in group 2. Reasons for delay were clinical instability in 6 babies, transfer to outpatient follow-up in 5 babies, neglect of family in 3 babies. First dose combination vaccine was delayed in 63 (45.6%) babies. Reasons for delay were clinical instability in 31 babies, doctor’s not recommended in 16 babies, neglect of family in 10 babies and fever of baby in 6 cases. Thirtysix percent of babies delayed in group 1 and 54.9 % of babies delayed in group 2. Conclusions: Healthy premature and low birthweight newborns should be vaccinated according to vaccination schedule at recommended chronological age for term babies. However in daily practice, these proposal can not be fully achieved. We suggest that with educational programs to care givers and families vaccination rates can be increased. Keywords: premature baby, vaccination [P-007] Introduction of an infant’s feeding disorder through a case study Ágnes Rita Martonosi, Éva Németh, Noémi Scheuring, László Szabó Heim Pal Children Hospital Background&Aims: Beside curing the somatic symptoms of patients with chronic diseases, leading a psychological support and a mental care to their families also play a major role during the healing procedure. Methods: Through a follow-up method we are introducing a treatment of an infant called Barnabas with multiplex congenital limb malformation having a cardiac surgery and feeding with nasogastric tube (NGT). While stabilizing his status we reached the exclusively oral feeding by paying attention not only to his physical but also his mental health condition. Results: After having multiplied heart failures caused emergency cardiac surgery as of eleven days old, Barnabas required long-term mechanical ventilation. Further care in our department began through feeding exclusively by NGT. After fighting infections, his vital parameters allowed attempting minimal oral feeding, still supplemental artificial nutrition was necessary. Becoming two months old, he started to refuse NGT by his body language or pulling out the tube, manifesting also repeated retching, vomiting, without detectable organic cause. Oral feeding was given during his arousal period, supplemented by NGT while resting. During our care we completed the treatment with a parentinfant consultation every day. Barnabas went home at three months old, with positive weight tendency. 1.5 months later, during a necessary re-hospitalization his attempts with putting all his power for pulling out his tube paired with oral underfeed, retching made his nourishment critical. Treating his infection, replenishing the required fluids and nutrition, continuing the parentinfant consultations, the willpower of Barnabas and persistent cooperation, patience and confidence of his parents allowed reaching the solely oral feeding. Conclusions: Creating a complex and long-lasting care for the patients with chronic diseases and their family members is challenging. Instead of focusing only the somatic symptoms we should apply to bio-psycho-social approach to reach an effective healing. Keywords: feeding disorder, parent-infant consultation, psychological support [P-008] Gitelman Syndrome Case Report Ahmet Ibrahim Bektasoglu, Burcu Karakayalı, Ismail Islek, Ahmet Sami Yazar, Julide Cavus Pediatric Clinic of Umraniye Training and Research Hospital, Istanbul, Turkey Context: Gitelman syndrome (GS) is an inherited autosomal recessive kidney disorder. It can diagnosed during adolescence or early adulthood based on clinical and biochemical findings. - 20 - Case Presentation: We present a case of 12 years old male, admitted our emergency service with rash and itching. Questioning revealed, patient had carpal spazm, hand paresthesia, increased thirst, frequent urination and nocturnal enurezis. Family never appealed for this further symptoms. Patient diagnosed parvovirus B19 infection and GS. Parvovirus B19 serology performed. Typical biochemical abnormalities for GS as hypokalemia, hypomagnesemia, hypocalciuria, metabolic alkalosis and hyperreninemic hyperaldosteronizm were detected. The patient was treated with oral potasium, magnesium and desmopressin, biochemical abnormalities and symptoms gradually regressed. Conclusion: Gitelman syndrome presentation and diagnosis is usually made during adolescence or early adulthood. It should be considered as a differential diagnosis of hypokalemia, especially in late childhood and adolescent age group, but must be noted that it can even occur under 6 years, also be noted that typical symptoms may not be the admitting reason. Keywords: Gitelman Syndrome, Hypokalemia, Metabolic acidosis [P-009] A Rare Cause Of Acute Kidney Failure Caused By Rabdomyolysis: Weil Disease Alkim Beyhan Akdeniz University School of Medicine, Pediatrics Deparment, Antalya, Turkey Leptospirosis is a commonly seen zoonosis caused by a bacteria called Leptospira. Even though Weil Disease is seen only in %5-10 of Leptospiral infections, because it affects a variety of organs it’s course is quite severe. An 18 year old male patient is presented with a greenish colored feces which had diarrheic characteristics. He had no fever but he noticed that his daily urine output has decreased. The patient had normal blood pressure(115 systolic, 80 diastolic mmHg). He had a 2cm hepatomegaly on the midclavicular line, periorbital and peripheral edema. His lab results showed that he had thrombocytopenia, an increase in creatinine, BUN, creatinine kinase and myoglobin levels.(Table 1) The second case was a 15 year old male patient who had had fever for 4 days, diffuse myalgia and muscle weakness. In his physical examination, he had diffuse jaundice, a 3cm hepatomegaly on the midclavicular line and crackles on the middle and inferior lobes of the right lung. Similar to the first patient, the second patient had thrombocytopenia, lymphocytopenia, an increase in creatinine, BUN and creatinine kinase levels. In addition to this he had conjugated hyperbilirubinemia. Upon further examination, both patients were found to have swam in the same river, approximately 10 days before the symptoms began. Because of the similar history, we studied microscobic agglutination test for Leptospira, which were both found positive(1/800 Leptospira Icterohaemorrhagiae for the former and 1/100 Leptospira Pomona for the latter) The first patient had 2 courses and the second one had 3 courses of hemodialysis and ultrafiltration. The first patient was administered meropenem and the second one meropenem and doxycycline. With both of these cases we wanted to emphasize that patients who were presented with acute kidney failure secondary to rabdomyolysis could have an infectious origin such as Leptospira. Keywords: Leptospirosis, Rabdomyolysis [P-010] Portal cavernoma in a child – case report Ancuta Ignat1, Vasile Valeriu Lupu1, Gabriela Paduraru1, Angelica Cristina Marin1, Bogdan Ciubara2, Marin Burlea1 1 Pediatrics Department, University of Medicine and Pharmacy “Gr. T. Popa”, Iasi, Romania 2 Anatomy Department, University of Medicine and Pharmacy “Gr. T. Popa”, Iasi, Romania Cavernous transformation of the portal vein (also called portal cavernoma) occurs when the native portal vein is thrombosed and it is replace the normal single channel portal vein with numerous tortuous venous channels. Cavernous transformation has been shown to form as early as 6 to 20 days after acute thrombosis of the portal vein. In about 30% of cases no underlying cause is found. It occurs much more commonly in patients without underlying liver disease. Often, it is associated with portal hypertension because the collateral veins are not able to adequately handle the splenic and mesenteric inflow. We report the case of a child of 13-year-old female presented with asthenia, headache, somnolence and hematemesis (an isolated instance 7 days prior to admission). Associated findings in this case included esophageal varices grade III, signs of splenomegaly (severe microcytic hypochromic anemia, leukopenia and thrombocytopenia). The diagnosis was confirmed with liver computerized tomography after obtaining a normal ultrasound. Better control of hemorrhagic and thrombotic complications in the patients with portal cavernoma substantially improves their life span and the clinical outcome. Whereas portal hypertension can in some cases be treated with transjugular intrahepatic portosystemic shunt (TIPS), but the absence of normal portal circulation usually makes this impossible. Keywords: portal cavernoma, esophageal varices, splenomegaly - 21 - [P-011] [P-012] Analysis of pediatric pancreatitis (APPLE). Multicenter prospective data collection and analysis by the Hungarian Pancreatic Study Group (HPSG) Observational Clinical Trial About Pain IN EArly Phase of Pediatric pancreatitis (PINEAPPLE) Andrea Parniczky1, Dóra Mosztbacher2, Natália Lásztity1, Anna Tóth3, Noémi Vass3, Judit Czelecz7, Csilla Andorka5, Gábor Veres5, Ildikó Guthy4, Veronika Ila4, Balázs Csaba Németh6, Péter Hegyi6 1 Heim Pál Children’s Hospital 2 János Balassa County Hospital, Department of Pediatrics, Szekszárd, Hungary 3 University of Szeged, Faculty of Medicine, Department of Pediatrics and Pediatric Health Center, 4 Szabolcs-Szatmár-Bereg County Hospitals, Jósa András University Teaching Hospital, Nyíregyháza, 5 Semmelweis University, First Department of Pediatrics and Pediatric Health Center, Budapest 6 University of Szeged, Faculty of Medicine, First Department of Medicine, Hungary 7 Bethesda Children’s Hospital, Budapest, Hungary Background&Aims: The incidence of pediatric pancreatitis(PP) has increased in the last decade. Some of the recent studies showed that the occurrence of the disease has grown over 10/100.000 which is not much less than in adults. We have established the Paediatric Section of the Hungarian Pancreatic Study Group in order to organize nationwide data collection and improve the management of the disease. Our aim was to analyse the epidemiology, risk factors, management and clinical outcome of PP in Hungary. Methods: 56 children suffering from PP were enrolled from 7 centres between 2012 and 2014. Results: 61% of the children were female. 31 acute(AP), 11 recurrent acute(RAP) and 14 chronic pancreatitis(CP) cases were recorded in the registry. 84% of the AP patients had mild and 16% moderate episodes, however, no severe AP was observed. In RAP patients pancreatitis seemed to be more severe than in patients having isolated episodes (mild: 73% moderate: 18%, severe: 9%). Mortality was not observed at all. Without genetic testing we could identify the etiological factors only in 44% of the cases, the others remained idiopathic.In 17 cases, genetic analyses of PRSS1, SPINK1, CFTR and CTRC genes have been completed. Genetic alterations in PRSS1 were found in 3 cases(all CP), in SPINK1 in 4 cases(1 RAP and 3 CP), in CFTR in 1 case(CP) and in CTRC in 10 cases(3 AP and 7 CP), In 5 CP patients mutations in two genes were observed(3 SPINK1-CTRC, 1 PRSS1-SPINK, 1 CFTR-CTRC). Conclusions: Genetic testing is essential to identify the etiological factors in children with pancreatitis. Keywords: pediatric pancreatitis, genetic testing, multicenter prospective trial Dóra Mosztbacher1, Andrea Párniczky2, Fanni Zsoldos2, Anna Tóth3, Veronika Ila4, Maisam Abu El Haija5, István Tokodi6, Boglárka Fehér7, Károly Bakó7, Dénes Molnár8, Andrea Szentesi9, Péter Hegyi9 1 Department of Pediatrics, János Balassa County Hospital, Szekszárd, Hungary 2 Heim Pál Children’s Hospital, Budapest 3 Department of Pediatrics and Pediatric Health Center, University of Szeged, Faculty of Medicine, Szeged, Hungary 4 Department of Pediatrics, Dr. Kenessey Albert Hospital, Balassagyarmat, Hungary 5 Division of Gastroenterology, Hepatology and Nutrition, Cincinnati Children’s Hospital Medical Center, Cincinnati, USA 6 Department of Pediatrics, Szent György Teaching Hospital of County Fejér, Székesfehérvár, Hungary 7 Department of Pediatrics, University of Debrecen, Faculty of Medicine, Debrecen, Hungary 8 Department of Pediatrics, University of Pécs, Faculty of Medicine, Pécs, Hungary 9 First Department of Internal Medicine, University of Szeged, Faculty of Medicine, Szeged, Hungary Background&Aims: The documented incidence of pediatric pancreatitis (PP) is very low, less than 1/100,000 in almost all European countries, whereas it is around 3.6-13.2/100,000 in the USA and Australia. Moreover, there are unexpectedly large differences between the incidences of PP among the countries and hospitals in Europe. The aim of the PINEAPPLE study (ISRCTN35618458) is to understand the current practice of diagnosis of PP, to demonstrate the difference of the incidence of PP between the various countries based on the pancreatic enzyme measurement(PEM) and to develop EBM guidelines that helps to evaluate (in a reliable and cost efficient way) the necessity of PEM and abdominal ultrasonography when a child has abdominal pain. Methods: PINEAPPLE is a registered, observational, multinational clinical trial. The PINEAPPLE-R subtrial is a retrospective review on children records appearing at ER units, whereas, the PINEAPPLE-P subtrial is a prospective part of the study where detailed patients data (concerning medical history, complaints, symptoms, physical examination) are collected, PEM and abdominal imaging are performed in all cases. Until now 23644 patients records/PINEAPPLE-R and 113 patients/ PINEAPPLE-P were enrolled from eight pediatric centres. Results: PINEAPPLE-R: 8,3% (1970/23644) of the patients appearing at ER unit had abdominal pain. Only 9,7% (192/1970) of them had PEM, whereas 30% (592/1970) had transabdominal ultrasonography. Pancreatitis was diagnosed in five cases only. In case where 21,6% (157/728) PEM were performed, the incidence of pancreatitis (4/728) was six times higher, than in case where just 2,8 % (35/1242) PEM were performed (1/1242). PINEAPPLE-P: 1 pancreatitis of 113 patients with abdominal pain was diagnosed. - 22 - Conclusions: The PINEAPPLE-R clearly shows that the number of PEM performed at ER units are unacceptably low in children, which could be the reason of low incidences of PP. More patients are crucially needed for PINEAPPLE-P in order to develop EBM guidelines. Keywords: pancreatitis, abdominal pain, EBM [P-013] An Infant with Congenitally Corrected Transposition; Case Report Aras Tulunoglu1, Osen Arı1, Helen Bornaun1, Aylin Öztarhan2, Murat Şahin1, Kazım Öztarhan1 1 Kanuni Sultan Suleyman Hospital, Pediatrics & Pediatric Cardiology Department, Istanbul, Turkey 2 Sisli Etfal Hospital, Gynegology and Obstetrics Department, Istanbul, Turkey Introduction: Congenitally corrected transposition of the great vessels (CCTGA) is a rare congenital heart defect associated with multiple cardiac morphological abnormalities and conduction defects. Case Description: One year old girl with no medical history was referred with tachypnea, dyspnea and tachycardia. Her height, weight and head circumference were between 10 to 25 percentile. No significant neuromotor and pulmonary abnormalities were detected. Initial ECG report showed narrow QRS complex supraventricular tachycardia. Rapid intravenous adenosine administered, after stabilization the ECG monitor revealed that the PR interval had shortened, a delta wave were present right before the QRS complex and Q waves were present in the right precordial leads but absent in the left precordial leads. The patient was suspected with supraventricular tachycardia and WolffParkinson-White syndrome associated with congenitally corrected transposition of the great arteries (CCTGA). Echocardiography had revealed ventricular inversion. The patient was prescribed propranolol. A Holter monitor is used for assessment of AV block and atrial arrhythmia, none had seen. A week later the patient had discharged for follow-up. Discussion: CCTGA is a rare defect that reported in 0.5% of patients with congenital heart disease. In situs solitus, the morphologic right ventricle is anatomically positioned to the left of the morphologic right ventricle. Blood flows in an effective sequence; however, the right ventricle supports the systemic circulation in this disorder. Prognosis depends on AV conduction, arrhythmias, structural abnormalities and degree of hemodynamic disturbance. Conclusion: Supraventricular tachycardia is the most common symptomatic dysrhythmia in infants and children and CCTGA should be considered. The diagnosis of CCTGA is usually made by echocardiography. ECG can be helpful to show the ventricular bundle branches are inverted and the initial activation is oriented from rightto-left. Keywords: congenitally corrected transposition, supraventricular tachycardia, Wolff-Parkinson-White syndrome [P-014] Isolated Left Ventricular Non-Compaction (LVNC) and Restrictive Cardiomyopathy with missense mutation in MYH7 gene; Case Report Kazım Öztarhan1, Şükrü Candan4, Tuğba Erener5, Helen Bornaun1, Aras Tulunoğlu2, Elif Yılmaz Güleç3, Akl Fahed6, Zeynep Ocak3 1 Kanuni Sultan Suleyman Education and Research Hospital, Department of Pediatric Cardiology Istanbul, Turkey 2 Kanuni Sultan Suleyman Education and Research Hospital, Department of Pediatrics, Istanbul, Turkey 3 Kanuni Sultan Suleyman Education and Research Hospital, Department of Medical Genetics, Istanbul, Turkey 4 Ataturk General Hospital, Department of Medical Genetics, Balikesir, Turkey 5 Maltepe University Faculty of Medicine, Department of Neonatology, Istanbul, Turkey 6 Harvard Medical School, Department of Genetics, Seidman Laboratory Introduction: Isolated left ventricular non-compaction (LVNC) is a myocardial disorder characterised by prominent ventricular trabeculations and deep recesses extending from the LV cavity to the subendocardial surface of the LV wall with or without LV dysfunction. Case Report: A-4 year-old girl with a history of respiratory problems was referred with palpitations, sweating, dyspnea and syncope. After the initial examination and treatment the patient was suspected for cardiac failure. Transthoracic echocardiography had revealed prominent ventricular trabeculations extending from the LV cavity, dilatation of the left atrium (LA) and moderate mitral valve insufficiency. On doppler tissue imaging, there was a diastolic dysfunction of the LV in restrictive pattern. MRI and cardiac catheterization had confirmed the trabeculations and restrictive pattern of the LV. The patient prescribed with diuretics and ACE inhibitors and added to cardiac transplantation list. Molecular analysis had performed to family members with target capture sequencing which is created for known cardiomyopathy genes group. The mutation in our case was a novel missense mutation located in exon 14 (GCA/GTA) A426V-MYH7 coding region. Segregation analysis within the family revealed that both parents and unaffected brother were homozygous wild genotype (GCA/GCA). Discussion: Isolated left ventricular non-compaction (LVNC) is a morphological abnormality of excessive trabeculation of the LV, often complicated by ventricular dysfunction, arrhythmias and cardioembolism. Screening of first-degree relatives is warranted, asymptomatic family members may have good prognosis. Some of the genetic mutations associated with LVNC include the G4.5 gene, DTNA (α-dystrobrevin), Cypher/ ZASP, lamin A/C cardiac troponin T (TNNT2), myosin heavy-chain gene (MYH7), cardiac actin (ACTC1), SCN5A and MYBPC3. Conclusion: LVNC is a form of cardiomyopahty that there are no evidince of significant difference from the - 23 - other forms of cardiomyopathies. In this case we report LV dysfunction in a restrictive pattern with a novel missense mutation of the MYH7 gene. Keywords: Isolated left ventricular non-compaction, restrictive cardiomyopathy, MYH7 gene [P-015] A case of primary immunodeficiency:Severe combined immunodeficiency Arzu Aras, Hatice Öztürk, Aynur Bedel, Zehra Esra Onal, Çağatay Nuhoğlu Haydarpaşa Numune Education and Research Hospital,Pediatri Department,Istanbul Background&Aims: Methods: Results: Conclusions: Primary immune deficiencies, depending on hereditary gene defects, associated with disorders in the functioning of the immune system with increased susceptibility to infections are characterized by the tendency to the formation of autoimmune and malign disorders. Combined immunodeficiency is called severe combined immunodeficiency due to genetic defects in various T-cell differentiation disorders occur as a result of fully blocked T-cell differentiation. 2-month old girl was admitted to our hospital with cough and fever.She had cough and fever during the last 20 days.With a diagnosis of pneumonia, she was treated with ceftriaxone and vancomycin in previous hospitalization. Because of no recovery of fever and cough with the current treatment, the patient was hospitalized in our clinic for further evaluation and treatment. She had no health problem after birth. Her family history showed epilepsy presence of her father, death of four brothers without any reason when they were approximately 2 months of aged, and the parents are the 1st degree cousin (uncle’s children). Lung infection in an early age and early age death of brothers due to no reason in history, immune deficiency was considered. Laboratory results showed the detection of lymphopenia and hypogammaglobinemia strengthened our diagnosis as primary immunodeficiency. Lymphocyte subsets; CD3 %6, CD3CD4 %3.85, CD3CD8 %2.29, CD19 %0.06, CD1656 %89,25 T(-) B(-), were detected respectively. She was diagnosed with severe combined immunodeficiency. Early diagnosis and treatment of primary immune deficiency, is very important in order to reduce the risk of death and complications. Therefore, early recognition of immune deficiency by clinicians is important for the prevention and reduction of mortality and appropriate treatment of complications due to recurrent infections. Keywords: Severe combined immune deficiency, children [P-016] septo-optic displasia syndrome caused by congenital cmv infection Asuman Kıral, İsmet Düşmez, Doruk Gül, Asena Pınar Sefer Goztepe Research Hospital Istanbul/Turkey Introduction: Septo-optic displasia syndrome( De Morsier Syndrome ),is a rare congenital malformation syndrome.Its prevelance is 1:50000 and has 3 characteristic speciality which are optic nerve atrophy(causes nistagmus),septum pellucidum agenesia, hypothalamic-pituitary disfunction and %50 cases have schizencephaly.Congenital CMV infection rarely causes this syndrome in embriological stage. In this case;we describe the importance of differential diagnosis in prolonged jaundice Case: 3 month aged patient who had jaundice,central hypotiroidism,hypotonia and hepatosplenomegaly,was sent to our hospital because suspecting metobolic disesase from another hospital. He had jaundice, hypotonia,rotatuar nistagmus,macrocephaly,2-3/6 cardiac murmur,hepatosplenomegaly in physical examination Laboratory results; WBC:7280/mm^3,PNL 25%,LYM 66%,Hb:9.73 g/dL,Hct: 27.6%,ALT:356 IU/L,AST:527 IU/L,GGT:70 IU/L,AF:796 IU/L,T.Bil:9.18 mg/dL,D. bil:5.96 mg/dL,electrolits and urinalyse was normal. Hemolytic anemia,viral hepatitis,aminoacid metabolic diseases,CMV infection,galactosemia,peroxysmal diseases,pituitary disfunction,biliary tract agenesia, Wolman disease,intracranial pathology was tought in differential diagnosis. Anti CMV IgG (+), IgM (-) was the only meaningful result in viral serology Furthermore, urinalysis for Galactosemia was negative,fitanic and pyristanic acid was negative(for peroxysismal diseases). The other methabolic labratory results was normal Secundum ASD was detected in ECHO. Lateral ventricules was enlarged in Cranial USG Hepatosplenomegaly and grade 1 hepatosteatosis was detected in abdominal USG.Surrenal glands were intact. Bilateral optic disc atrophy was realized in ophtalmologic examination. Pediatric Endocrinology detected panhypopituitarsm as a result of further investigation. BERA test for audition was normal. Pediatric Gastroenterology advised liver biopsy and the result was neonatal hepatitis. In Cranial MRI; right frontal lobe closed lib schizencephaly,septum pellicidum agenesia was detected. His CSF CMV PCR<79.7copy/ml,Urine CMV PCR:19434copy/ml The results were meaningful for Congenital CMV Infection and Pediactric infectious disease advised ganciclovir treatment for 6 weeks. The baby who has bilateral optic disc hipoplasia, septum pellucidum agenesia, shizencephaly, CMV infection, rotatuar nistagmus, panhipopituitarism was diagnosed to Septo-optic displasia Syndrome. - 24 - [P-018] Keywords: septo-optic displasia syndrome, congenital cmv infection Isolated exocrine gland involvement associated with mutation of 1677 delta heterozygote and M470V heterozygote polymorphism on the CFTR Gene [P-017] Syndromic Severe Congenital Neutropenia: JAGN1 Mutation Avniye Kübra Baskın, Funda Çipe, Çiğdem Aydoğmuş, Gonca Keskindemirci, Kaan Boztug Division of Pediatric Allergy and Immunology, Kanuni Sultan Suleyman Research and Training Hospital,Istanbul,Turkey Severe congenital neutropenia (SCN) is characterized by susceptibility to recurrent life threatening bacterial infections due to maturation arrest of neutrophils. Different studies have showed mutations in ELA 2, HAX 1, G6PC3, WAS, GF11 and VPS45 genes. Jagunal homolog 1 (JAGN 1) mutations were recently defined as a rare genetic cause severe congenital neutropenia defect in 2014. Our patient was a 10 years old male born from first degree cousin parents at 1900 gram birth weight. He was admitted due to neonatal sepsis for a month at fourth day of postnatal age. He was re-hospitalized because of wounds on bilateral lower extremities and severe neutropenia has been noticed. Bone marrow aspiration revealed maturation arrest of neutrophils. He was started to treat with G-CSF (5 µg/kg) with the initial diagnosis of Kostmann syndrome. Physical examination was not notable except triangular face and extrovert ears. Initial laboratory evaluation revealed only neutropenia. Despite treatment he had recurrent infections including pneumonia, otitis, sinusitis and skin abscess even pulmonary tuberculosis. In addition, he suffered from asthma due to house dust mite allergy. Mutation analysis for common mutations for SCN were found to be negative. After identification of JAGN1 deficiency, it was sequenced the relevant gene and detected a homozygous missense mutation in exon 2 of JAGN1 gene (c 130 c>T, p. His 44 Tyr). The patient is still followed up and receiving high dose G-CSF(10 µg /kg). Matched unrelated donor screening is continuing for bone marrow transplantation because of lack of family matched donor. In conlusion, JAGN 1 mutations must be considered in patients with severe congenital neutropenia especially with facial dismorphism and multisystemic disorder. Keywords: JAGN1 mutation, Severe congenital neutropenia Ayla Balcı1, Duygu Övünç Hacıhamdioğlu2, David Terence Thomas3, Ayça Sözen1, Arif Kut4, Ismail Göçmen1 1 Department of Paediatrics, Faculty of Medicine, Maltepe University 2 Department of Pediatric Nephrology, Faculty of Medicine, Maltepe University 3 Department of Pediatric Surgery, Faculty of Medicine, Maltepe University 4 Department of Pediatric Pulmonology, Faculty of Medicine, Maltepe University Introduction: CF is a genetic disease that commonly involves the lungs, gastrointestinal system, pancreas and exocrine glands. It is rare for a patient to have normal lungs yet other system involvement. Herein we report a patient affected by only exocrine gland dysfunction presenting with anemia due to cystic fibrosis (CF). Case: A 2.5 month-old boy was born meconium stained from unconsanguineous healthy parents at 38th weeks of gestational age, weighing 2600 g. Prenatal history was insignificant. Family history revealed Cystic Fibrosis (CF) in his mother’s cousin (exocrine involvement, female) and father’s cousin (exocrine and lung involvement, male). The patient was admited to our hospital at 2.5 months of age (weight: 6300 g. 50th percentile, height: 61 cm, 50th percentile) due to weakness, facial edema and paleness. Deep anemia (hemoglobin 6 g/dl) was detected, requiring recurrent erythrocyte transfusions. Hypokalemic metabolic alkalosis was found during investigation of etiology of anemia. There was no renal potassium, calcium or sodium loss. Analysis of blood and urine was evaluated normally. Sweat chloride test results were 68 mmol/L, 92 mmol/L and 98 mmol/L (normal: <60 mmol/L). A diagnosis of CF was made. Vitamins A, D, E and K, plus potassium, and pancrelipase supplementation was commenced and his anemia resolved. CFTR gene analysis was performed on 10th exon and heterozygote mutation of 16677delta (M470V polymorphism) was detected. The patient currently has no signs of lung involvement and his neurological development is appropriate for his age. Discussion: It is rare for CF to effect other systems without lung involvement. We report a small infant where CF presented with anemia, exocrine gland dysfunction but no lung involvement. Heterozygote polymorphisms are not known to lead to disease. However, interestingly, our patient’s 1667delta heterozygote mutation (M470V polymorphism) seems to have led to CF. Further genetic analysis has been planned. Keywords: cystic fibrosis, deep anemia, infant - 25 - [P-019] Viral Encephalitis With Atypical and Fulminant Prognosis (Case Report) Aynur Bedel, Hatice Öztürk, Tamay Gürbüz, Dilşad Koca, Çağatay Nuhoğlu Haydarpaşa Numune Eğitim ve Araştırma Hastanesi Encephalitis in childhood can be occured fulminant and mortal. Permanent morbidity is present even over 35% in treated patients. In this study atypical encephalitis which is compatible with radiological encephalitis, but can not be determined by serologically active fulminant course and get benefit by acyclovir treatment with antibiotics, steroids and IVIG is presented. Keywords: Viral encephalitis, atypical, fulminant [P-020] Anti N Methyl D Aspartate(NMDA) reseptor encephalitis after healing of herpes simplex virus (HSV) encephalitis Aynur Guliyeva1, Türkan Uygur Şahin2, Ayşegül Doğan Demir1, Akın İşcan2 1 Bezmi Alem Üniversitesy Medical Faculty, Paediatrics 2 Bezmi Alem Üniversitesy Medical Faculty, Children Neurology Anti NMDA (N-methyl D-aspartate) receptor antibody encephalitis caused by an autoimmune reaction of the NDMA receptor. Sometime this disorder relapses after herpes simplex virus1 (HSV1) encephalitis. Anti NMDA (N-methyl D-aspartate) receptor antibody encephalitis have been hipothesied to be secondary to postviral autoimmunity. 25 month old girl was admitted with a fever, seizures, agitation. Prenatal and postnatal history wasn’t signifancy. No family history feature. Acute phase reactants were negative and other laboratory tests were normal range. Cranial magnit resonant image (MRI) is compatible with herpes encephalitis: bilateral themperoparietal edema were found. In cerebrospinal fluid HSV 1DNA-PCR were positive. Spike waves were seen in EEG. Because of these findgins acyclovir treatment were applied for 3 weeks. After 10 days from discharge in patient began speech disorder, gait disturbance, choreiform movies, oropharingeal dyskinesia. With the diagnosis of limbic encephalitis began IVIG therapy. At the same time sent cerebrospinal liquid antiNMDA receptor antibody was positive. Admitted pulse steroid therapy. Seven times plasmapheresis treatment - refractory patients began cyclophosphamide therapy. After receive 2 dose of cyclophosphamide treatment response were taken. Gradually improved swallowing and and disappeared dyskinesia and choreiform movies. Control cranial MRI was compatible with anti NMDAR antibody encephalitis (cerebellar and cerebral volume loss and ventricular dilatation). The treatment of patients is continuing. Treated patients with encephalitis and after develop choreiform movie, agitation and dyskinesia must come to mind NMDA encephalitis. Treatment options is IVIG, pulse steroid, plasmapheresis and cyclophosphamide. Keywords: Anti NMDA (N-methyl D-aspartate) receptor antibody encephalitis, oropharıngeal dyskinesia, choreiform movies [P-021] α-Melanocyte-Stimulating Hormone and Agouti-Related Protein: Do They Play a Role in Appetite Regulation in Childhood Obesity? Aysel Vehapoğlu1, Serdar Türkmen2, Şule Terzioğlu3 1 Department of Pediatrics, Bezmialem Vakıf University, School of Medicine, Istanbul, Turkey 2 Department of Biochemistry, Gaziosmanpaşa Taksim Training and Research Hospital, Istanbul, Turkey. 3 Department of Medicinal Biology, Bezmialem Vakıf University, School of Medicine, Istanbul, Turkey. Background&Aims: The hypothalamus plays a crucial role in the regulation of feeding behavior. The anorexigenic neuropeptide α-melanocyte-stimulating hormone (α-MSH) and the orexigenic neuropeptide agouti-related protein (AgRP) are among major peptides produced by hypothalamus. This study investigated the plasma concentrations of α-MSH and AgRP in underweight and obese children and their healthy peers, and the association between α-MSH and AgRP levels and anthropometric and nutritional markers of malnutrition and obesity. Methods: Subjects aged 2 to 12 years were divided into 3 groups:57 underweight (due to loss of appetite and less frequent hunger episodes) prepubertal children, 61 healthy obese children, and 57 healthy age and sex-matched children of normal weight. Plasma fasting concentrations of α-MSH and AgRP were measured by enzyme-linked immunosorbent assay. The relationships between plasma concentrations of α-MSH and AgRP and anthropometric data, serum biochemical parameters, and homeostatic model assessment of insulin resistance were also evaluated. Results: Obese children had significantly lower α-MSH levels than did underweight (1194±865 vs. 1904±1312 ng/ml, p= 0.006) and normal-weight (1194±865 vs. 1762±1463 ng/ml, p= 0.036) children; there were no significant differences in the α-MSH levels between the underweight and normal-weight children (p= 0.811). Also, no significant differences were observed between the underweight and obese children in the AgRP levels (742±352 vs. 828±417, p=0.125). We found a significant positive correlation between the plasma α-MSH and AGRP levels (p< 0.001) and a negative correlation between the α-MSH levels and weight z-score (p= 0.017), BMI (p= 0.019), BMI z-score (p= 0.014), BMI percentile (p= 0.017), insulin (p= 0.045), and CRP (p< 0.001). Conclusions: This study is the first to demonstrate body weight-related changes in α-MSH and AgRP levels in children. Circulating plasma α-MSH levels in obese children were markedly lower than those of underweight and normal-weight children. This suggests that α-MSH - 26 - [P-023] could play a role in appetite regulation. Keywords: α-MSH, AgRP, underweight children [P-022] Role of the circulating adipocytokines vaspin, apelin, and visfatin in loss of appetite in underweight children: A pilot trial Aysel Vehapoğlu1, Feyza Ustabas1, İlker Tolga Özgen2, Şule Terzioğlu3, Banu Çermik1, Ömer Faruk Özer4 1 Department of Pediatrics, Bezmialem Vakıf University, School of Medicine, Istanbul, Turkey. 2 Department of Pediatric Endocrinology, Bezmialem Vakıf University, School of Medicine, Istanbul, 3 Department of Medicinal Biology, Bezmialem Vakıf University, School of Medicine, Istanbul, Turkey. 4 Department of Biochemistry, Bezmialem Vakıf University, School of Medicine, Istanbul, Turkey. Background&Aims: In anorexic children who eat slowly, consume a limited number of foods, lack interest in food, display an irregular eating pattern, and experience loss of appetite and less frequent hunger episodes, body weight and fat are reduced. Children require sufficient nutrients to support the immune system, and to help the body maintain health and normal bodily functions. Present study aims to investigate serum concentrations of vaspin, apelin, and visfatin in underweight children and their association with anthropometric and nutritional markers of malnutrition. Methods: We recruited 44 underweight prepubertal children (youngest age = 2 years) with thinness grades of 1, 2, and 3, and body weights <90% of ideal body weight due to loss of appetite and less frequent hunger episodes. Forty-one healthy age- and gendermatched controls were also included in the study. Serum fasting vaspin, apelin, and visfatin concentrations were measured by enzyme immunoassay. Results: Mean vaspin and apelin levels in the malnourished group (0.44 ± 0.18 and 483.37 ± 333.26 pg/mL, respectively) were significantly lower compared to healthy controls (0.58 ± 0.35 and 711.71 ± 616.50 pg/m, P = 0.024 and P = 0.041, respectively). No significant group differences were observed in visfatin levels (malnourished group: 177.81 ± 158.01 pg/mL; control group: 221.15 ± 212.94 pg/mL, P = 0.119). Vaspin and apelin levels were positively correlated with BMI z-scores (r = 0.297, P = 0.007; and r = 0.267, P = 0.016, respectively) in the malnourished group. Conclusions: In underweight children, decreased vaspin and apelin levels should be considered in the etiology of anorexia. Our data suggest that plasma apelin and vaspin levels represent a sensitive index of nutritional status that reflects changes in the fat levels of underweight children. Keywords: appetite regulation, vaspin, apelin Reduced antioxidant capacity and increased subclinical inflammation markers in prepubescent obese children and their relationship with nutritional markers and metabolic parameters Aysel Vehapoğlu1, Serdar Türkmen2, Nilüfer Göknar1, Ömer Faruk Özer3 1 Department of Pediatrics, Bezmialem Vakıf University, Faculty of Medicine, Istanbul, Turkey. 2 Department of Biochemistry, Gaziosmanpaşa Taksim Training and Research Hospital, Istanbul, Turkey. 3 Department of Biochemistry, Bezmialem Vakıf University, School of Medicine, Istanbul, Turkey. Background&Aims: There are associations between some inflammatory and oxidative markers and obesity in adults, but whether prepubescent children of different weights also have such markers has not been studied. We investigated multiple inflammatory markers and levels of erythrocyte oxidant/antioxidant enzymes in prepubescent children of different weights. Methods: Children aged 2–11 years were divided into three groups: 80 were underweight, 90 were obese but otherwise healthy, and 80 were healthy age- and sex-matched children of normal weight. We analyzed inflammatory markers and the total oxidant status, total antioxidant status, and total thiol level were also determined, and the oxidative stress index was calculated as an indicator of the degree of oxidative stress. Results: The obese group exhibited higher levels of fasting glucose, insulin, total cholesterol, triglycerides, the homeostatic model assessment of insulin resistance, and the homeostatic model assessment of β-cell function, CRP, neutrophils, and neutrophil/lymphocyte ratio, as well as lower TAS and total thiol levels than the other two groups (all p< 0.001). Moreover, TAS and total thiols were negatively correlated with age in the obese group (r= -0.212, p=0.001; r= -0.231, p<0.001, respectively). CRP levels in plasma were positively correlated with the body mass index (BMI), insulin and glucose levels, HOMA-IR, HOMA-β, WBC and neutrophil counts, and the NLR, and were negatively correlated with TAS and total thiol levels in the overall studied population. Conclusions: The coexistence of increased obesityrelated subclinical inflammation and decreased antioxidant capacity can be observed even in prepubescence, and may eventually increase the risk of long-term vascular damage. Keywords: Inflammation markers, Oxidative/ antioxidative status, Prepubescent children - 27 - [P-024] Clinical significance of human metapneumovirus in refractory status epilepticus and encephalitis: case report Aysel Vehapoğlu1, Özden Türel2, Türkan Uygur Şahin3, Nurettin Onur Kutlu4, Akın İşcan3 1 Department of Pediatrics, Bezmialem Vakıf University, Faculty of Medicine, Istanbul, Turkey. 2 Department of Pediatrics Infection, Bezmialem Vakıf University, Faculty of Medicine, Istanbul, 3 Department of Pediatric Neurology, Bezmialem Vakıf University, Faculty of Medicine, Istanbul, Turkey. 4 Department of Pediatric Emergency, Bezmialem Vakıf University, Faculty of Medicine, Istanbul, Turkey. Human metapneumovirus (hMPV) is a common cause of respiratory tract infections in children. Here we describe an infant with refractory status epilepticus and encephalitis in whom hMPV was detected in respiratory samples. A previously healthy 4-months-old male infant presented with ongoing seizure activity. He had 3 days history of mild rhinorrhea and suddenly experienced twitching at right extremity and eye deviation to the right. The seizure was unresponsive to rectal diazepam and could only be controlled by bolus midazolam and phenytoin infusion. His EEG tracing was consistent with multifocal epileptiform activity. Cerebrospinal fluid (CSF) examination showed 100 leukocytes/mm3 (all lymphocytes), glucose 51 mg/dl, and protein 24 mg/dl. Acyclovir and ceftriaxone were initiated pending culture results. Cranial magnetic resonance imaging showed no abnormalities. No bacterial growth was detected in blood, urine, respiratory, and CSF cultures. Investigation of viral pathogens on nasal scrapings by reverse transcription PCR revealed hMPV. CSF herpes simplex type 1 and 2 PCR were negative. He had no seizures during the next days and was discharged on the 25th day of admission. A broad spectrum of neurologic disorders ranging from seizures to fatal encephalitis/encephalopathy, have been reported in association with upper respiratory tract disease due to hMPV. Given the increasing evidence that hMPV may be one of causative agents rather than an incidental finding, we suggest that it should be tested along with other pathogens in children with status epilepticus and encephalitis. Keywords: Human metapneumovirus, refractory status epilepticus [P-025] Malnutrition in Spina Bifida Banu Yazici1, Busra Duzyol2, Huseyin Canaz3, Nursu Kara4, Ibrahim Alatas3 1 Department of Pediatrics, Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey 2 Department of Dieticianary, Florence Nightingale Hospital, Istanbul, Turkey 3 Department of Neurosurgery, Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey 4 Department of Neonatology, Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey The aim of this research is to determine if there’s any difference between malnutrition in children with spina bifida and normal population. The study included 112 girl and 129 boys in total 241 children with spina bifida whom we tracked their height, weight and head circumference in our clinic and we compared their results against the same age healthy children’s percentil. We found that the Spina Bifida Group is always get behind of healthy children percentil. The gap starts from 0-3 mounths and it grows with time. Even Spina Bifida Group’s and control group’s social and economic statue are same, there is a significant percentil gap (p<0.05). There’s significant gap between control group and children with spina bifida. Measurements of SB group is always behind the control group and lag increases with age. That’s why we declare the lag in percentil of children with spina bifida is not only based on malnutrition but also their condition. Keywords: Spina bifida, malnutrition, growth percentil [P-026] False-positive pneumococcal antigen test in a case of pediatric pleural empyema Bendavid Matthieu1, Caseris Marion1, Cointe Aurélie2, Mariani Patricia2, Pierron Charlotte3, Bidet Philippe2, Bonacorsi Stéphane4 1 AP-HP, General Paediatrics, Robert-Debré hospital, F-75019 Paris, France 2 AP-HP, Microbiology laboratory, Robert-Debré hospital, F-75019 Paris, France 3 AP-HP, Intensive Care unit, Robert-Debré hospital, F-75019 Paris, France 4 Paris Diderot University, Sorbonne Paris Cité, IAME, UMR 1137, F-75018 Paris, France Background&Aims: empyema is a serious complication of pediatric pneumonia requiring a rapid diagnosis of the causative agent for effective treatment and good outcome. The BINAX NOW® Streptococcus pneumoniae Antigen Card is an in vitro rapid and simple test used for the detection of S. pneumoniae soluble antigen and may be used directly on the pleural fluid. We report a falsepositive pneumococcal antigen test in pleural fluid of an 8-year’s old with pleural empyema following aspiration pneumonia. Case report: an 8-year-old girl was admitted in intensive care unit for fever, dyspnea and sepsis evolving for 24 hours. Medical history included encephalopathy due to hypoxic-ischemic brain injury, and respiratory insufficiency managed by tracheostomy. Investigations showed right empyema. Gram examination on the pleural fluid was negative but the S. pneumoniae antigen test performed on it was positive. The patient therefore received Amoxicillin. Cultures, mass spectrometry and 16S rRNA gene amplification then identified Parvimonas micra, Eikenella corrodens and Fusobacterium nucleatum. Microbiological analysis: we separately tested Parvimonas micra and Eikinella corrodens isolates from the pleural fluid with the BINAX NOW® S. pneumoniae test. As we didn’t isolate the strain of Fusobacterium nucleatum, we challenged another strain of Fusobacterium nucleatum from our collection. We observed a falsepositive in vitro test for Parvimonas micra and a negative test for Eikenella and Fusobacterium species. Finally, we performed specific pneumococcal polymerase - 28 - chain reaction on the pleural fluid that proved negative eliminating a co-infection with this pathogen. Consequently, the false-positive result for our patient was due to the presence of Parvimonas micra in the pleural fluid. Conclusions: clinicians should be aware that BINAX NOW ® S. pneumonia test performed on fluid from pleural infection may be falsely positive in case of anaerobic infection including Parvimonas micra and should interpret with caution this test result in context compatible with aspiration pneumonia. Keywords: empyema, false-positive pneumococcal antigen test, Parvimonas micra [P-027] A rare case of prenatally diagnosed galen vein aneurysm Betül ÇINAR1, Royala Babayeva1, Mine Özdil2, Ersin Ulu2, Yıldız Perk2, Mehmet Vural2, Civan Işlak3 1 İ.Ü Cerrahpasa Medical Faculty Department of Pediatrics, 2 İ.Ü Cerrahpasa Medical Faculty Department of Pediatrics, Neonatalogy, 3 İ.Ü Cerrahpasa Medical Faculty Department of Neuroradiology, Istanbul, Turkey Background&Aims: Galen vein aneurysms are rarely seen intracranial malformations which develop as a result of the presence of an arteriovenous fistula between choroidal branches of the cerebral artery and/or branches of the basilar artery and the dilated veins of the mesencephalon. Aneurysm of Galen vein malformation (VGAM) is the most common cerebrovascular malformation symptomatic in neonatal and infantile periods. Children with VGAM presenting during the neonatal period have a worse prognosis. Clinical manifestations include high-output congestive heart failure, bruit on head auscultation, hydrocephalus and drug-resistant seizures. Better clinical results and cognitive function are achieved with endovascular interventions to occlude interweaving vessels. Methods: A term, male baby whose prenatal ultrasound exposed 11mm Galen vein aneurysm, was born with cesarean section and admitted to the neonatal intensive care unit. The baby was active neurologically, anterior fontanelle was normal on palpation but a systolodiastolic bruit was notable on auscultation. On cardiac examination a 2/6 systolic murmur was audible. The cranial ultrasonography and MR venography detected a vascular malformation in proximity to quadrigeminal cysterna (13,6 mm). The echocardiography uncovered right ventricular hypertrophy, PDA, pulmonary hypertension and atrial septal defect. On the second day, the patient developed tachycardia, tachypnea and hypotension and treatment for heart failure was started. On the 6th day, endovascular embolisation was performed through transarterial approach. After the procedure, his hemodynamics gradually improved, cardiomegaly diminished but the patient developed generalized clonic convulsions, responsive to anticonvulsive treatment. On the 32nd day he was discharged with normal neurological, cardiac findings and breastfed. Results: Galen vein aneurysm is a rare, prenatally recognizable malformation which must be kept in mind in a neonate presenting with early onset high-output heart failure and seizures. Endovascular treatment seems to be the best primary treatment for VGAM. Excellent long-term cognitive and functional outcomes after endovascular occlusions of VGAM are reported. Keywords: Galen vein, heart failure, seizure [P-028] Complications with Rotavirus: Is it worth vaccination? Bilge Aldemir Kocabaş1, Adem Karbuz1, Halil Özdemir1, Anıl Tapısız1, Nurşen Belet1, Haluk Güriz2, Ergin Çiftçi1, Erdal Ince1 1 Division of Pediatric Infectious Diseases, Department of Pediatrics, Faculty of Medicine, Ankara University, Ankara, Turkey 2 Department of Clinical Microbiology, Faculty of Medicine, Ankara University, Ankara, Turkey Background&Aims: Rotavirus gastroenteritis (RG) causes severe fluid and electrolyte imbalance and development of fatal complications during the course of the disease, if untreated. Herein, we evaluated the clinical characteristics, incidence and types of the complications during RG in 276 patients in this retrospective study. Methods: We evaluated a total of 276 patients (164 male, 112 female) diagnosed with RG between January 2006 and February 2013 at the department of Pediatric Infectious Disease. Results: Male to female ratio was 1.5 and the median age at admission was 12 months (1-102 months). None of the patients had a history of Rotavirus immunization. While overall complication rate was 44.2%, the most common complication related RG was electrolyte imbalance and the second common complication was septicemia (32.6% and 6.5%, respectively). Klebsiella, E. coli and Candida species were the most common isolated microorganisms from the blood cultures. Conclusions: RG is still an important cause of morbidity in children which can cause serious complications such as sepsis. Lack of rotavirus vaccine in our national immunization program might contribute the RG related complications and prolonged hospitalization, health care expenses, family work loss, as well. Keywords: complications, Rotavirus, septicemia - 29 - [P-029] [P-030] Bilge Aldemir Kocabaş1, Adem Karbuz1, Ergin Çiftçi1, Fırat Beğde2, Selver Ametoglou2, Ali Adil Fouad3, Ayşe Kalkancı3, Zeynep Ceren Karahan4, Derya Aysev2, Erdal Ince1 1 Division of Pediatric Infectious Diseases, Department of Pediatrics, Faculty of Medicine, Ankara University, Ankara, Turkey. 2 Department of Pediatrics, Faculty of Medicine, Ankara University, Ankara, Turkey. 3 Department of Medical Microbiology, Faculty of Medicine, Gazi University, Ankara, Turkey 4 Department of Medical Microbiology, Faculty of Medicine, Ankara University, Ankara, Turkey Background&Aims: Kerion celsi (KC) is inflammatory form of tinea capitis. Herein, we report a ten year-old boy who had KC related to Trichosporon asteroides and successfully treated with fluconazole within eight weeks. This is the first KC case in the literature due to T. asteroides to our knowledge. Fatih Çelmeli1, Bilge Aldemir Kocabaş2, Ömer Bek3, Kemal Gökkuş4 1 Department of Pediatric Allergy-Immunology, Antalya Education and Research Hospital, Antalya, Turkey 2 Department of Pediatric Infectious Diseases, Antalya Education and Research Hospital, Antalya, Turkey 3 Department of Pediatrics, Memorial Hospital, Antalya, Turkey 4 Department of Orthopaedics, Memorial Hospital, Antalya, Turkey Background&Aims: Osteomyelitis caused by Salmonella typhi is rare in patients with no haemoglobinopathies or immunodeficiency diseases. There have been a few reports on septic arthritis and osteomyelitis in the humerus secondary to Salmonella infection in children. Trichosporon asteroides: A novel ethiological agent of Kerion celsi in a child Salmonella typhi septic arthritis and osteomyelitis in an infant Results: A 10-year-old boy was admitted to our hospital with complaints of swelling, discharge and pain behind of the right ear. There was no trauma history and initial physical examination revealed a painful and yellow colored purulent discharge and hair loss at an about 3x2 cm area on the scalp behind the right ear. There was also a mobile, painful palpable lymphadenopathy in the postauricular region. White blood cell count and acute phase reactants were within normal ranges. The skin lesion was consistent with KC and intravenous fluconazole (10 mg/kg/day) was initiated taking into account his previous history of terbinafine treatment. Also, ketoconazole shampoo was given two days a week. The reduction of pain and erythema was observed on the lesion within the first three days of treatment. Discharge was significantly reduced at the end of the first week and incrustation was observed on the lesion surface. He did not have fever episodes during the follow-up. Trichosporon colonies were detected on Sabouraud Dextrose Agar (SDA) plates. DNA sequencing was additionally performed for the molecular identification Trichosporon isolate. The patient was discharged on the 8th day of intravenous treatment with oral fluconazole (4 mg/kg/day) next week. The lesion was found to be significantly resolved at the control visit. At the end of the 8th week, new hair formation was observed on the lesion and the treatment was stopped. Results: An 11-montn-old male infant was referred to us with complaints of prolonged diarrhea, fever, and pain, swelling and restricted movement of left shoulder. He was born full term neonate and her parents were not consanguineous. He had no a history of any signs of immunodeficiency, sickle-cell disease or trauma. On his physical examination, he had fever (39º C), swelling and restriction of movement of the left shoulder. Laboratory tests revealed: absolute neutrophil count 6,160 /mm3, absolute lymphocyte count 4,900/mm3, absolute monocyte count 1,250/mm3, CRP 137 mg/dl, sedimentation rate 114 mm/hr. IgG 695 mg/dl, IgM 110 mg/dl, IgA 77 mg/dl, IgE 96 kU/L, anti Hbs 195 mg/dl. Flow cytometric evaluation of lymphocyte subsets were as following: CD3 59%, CD4 34%, CD8 18%, CD19 35%, CD20 35%, CD16-56 3%, HLA-DR %34. Pus and blood cultures yielded Salmonella. HIV was negative. NBT was positive. Hemoglobin electrophoresis was normal. Ultrasonography revealed left shoulder joint effusion. Radiographs and magnetic resonance imaging of the left shoulder was taken and showed osteomyelitis in humerus and the patient was referred to the orthopedic surgeon. Aspiration was performed which revealed 200 segmented WBCs per high-power field. Intraoperative left shoulder arthrotomy with irrigation, debridement, and a synovial biopsy were performed. The joint aspirate culture was subsequently positive for Salmonella. The species was later identified as Salmonella serotype typhi. The organism was susceptible to ampicillin, cefotaxime, and trimethoprim/sulfamethoxazole. Parenteral ceftriaxone was initiated. Conclusions: Differential diagnosis with bacterial pyoderma, abscess may avoid unnecessary and inappropriate surgical drainage. Keywords: Kerion celsi, tinea capitis, Trichosporon asteroides Conclusions: We report a case of a previously healthy infant who diagnosed with Salmonella typhi osteomyelitis of humerus. We think that possible molecular mechanism might be IL 12Rβ1 or IL 12p40 mutation. Keywords: osteomyelitis, Salmonella typhi - 30 - [P-031] Atypical Sturge-Weber syndrome Borbala Zsigmond1, Beata Rosdy1, Katalin Kollar1, Eva Kovacs2, Gyorgy Varallyay3, Marton Hernadi1 1 Neurology Department, Heim Pal Children Hospital, Budapest, Hungary 2 CT and Interventional Radiology Department, Heim Pal Children Hospital, Budapest, Hungary 3 MRI Research Center, Semmelweis University, Budapest, Hungary Introduction: Sturge-Weber syndrome is a rare, congenital neuro-cutaneous disease, which, in typical cases, contains one-sided facial port-wine stains, ipsilateral ocular lesions, leptomeningeal angiomas and epilepsy. However, in atypical cases these abnormalities can be found without dermal lesions, as well. The Case: 7- year-old child has been treated with epilepsy since the age of 6 month. For the neonatal focal seizures,which were not controlled sufficiently with carbamazepine alone, valproate was added to therapy. After three years without manifest seizures the medical treatment was gradually tapered during the next two years. After one-and-a-half year without anticonvulsant medication the patient experienced cumulative habitual seizures during a febrile infection at home, ending up in status epilepticus with focal initiation in our hospital. Parenteral midazolam was only temporarily effective, so intravenous valproate loading was successfully applied, and it was later per oral administered. The postictal EEG showed serious slowing in the right parieto-occipital region. Because of the possible infectious trigger of the seizures, emergency cranial MRI was performed with a suspicion of parainfectious encephalitis. The MRI showed significant atrophy of the right parieto-occipital region, and a streak signal change was observed between the cortex and the white matter in the same region. The lesion in question was identified as calcification or subacute hemorrhage. Cranial CT confirmed it as calcification and verified the diagnosis of Sturge-Weber syndrome. Conclusion: This case points out that the phakomatoses could present themselves even without the characteristic cutaneous abnormalities. Keywords: Neurology, Phacomatosis, Epilepsy [P-032] Imcomplete Kawasaki Disease in a twomonth old infant Burcu Karakol1, Burcu Karakayalı1, Burak Deliloğlu1, Taner Yavuz2, Şirin Güven1, İsmail İşlek1 1 Ümraniye Eğitim ve Araştırma Hastanesi, İstanbul 2 Zeynep Kamil Kadın ve Çocuk Hastalıkları Eğitim ve Araştırma Hastanesi, İstanbul Introduction: Kawasaki disease (KD) is an acute febrile multi-systemic vasculitis of unknown etiology. The incidence rate is very low in patients younger than 3 months of age. We reported here an infantile case of incomplete KD aged two months. Case: A two- month old infant with fever, and mild diarrhea for three days was admitted to the emergency department. Physical examination revealed polymorphic diffuse maculopapular rash and erythema on trunk and both upper extremities, he had oropharyngeal erythema, nonpurulent conjunctivitis on both eyes, a unilateral cervical lymphadenopathy measuring 1.5 cm. His initial laboratory tests included elevated CRP (7.5 mg/dl), no leukocytosis, hemoglobin of 9.4 g/dl, 440,000/µL platelets. Tests for TORCH infections were negative. He was admitted to the hospital. On day four, he had still fever, diarrhea and rash, tests were repated. A drop of hemoglobin (7. g/dl) and mild thrombocytosis, hypoalbuminemia, elevated erythrocyte sedimentation rate (ESR) (78 mm/hr), elevated fibrinogen were noted. Cultures remained sterile and antibiotics were stopped. The patient underwent echocardiography; showing a saccular dilated LCA and LAD. Intravenous immunoglobuline 2 g/kg and aspirin 60 mg/kg/day were given on the seventh day of fever. Fever dropped and the rash disappeared dramatically with treatment. At day 13, laboratory tests showed thrombocytosis (620,000/ µL) and elevated ESR (67 mm /hr). Control echo on the 14th day showed amelioration of findings and aspirin dose was lowered to antitrombotic dosage. The third echo on the 35th day of disease showed normal coronary vascular anatomy. Echocardiography on the second month remained normal. Conclusion: Because of higher risk for developing coronary artery abnormalities (CAA), precise diagnosis of KD is important. KD should be considered in the differantial diagnosis of all febrile ilnesses in infants where the fever persists for more than 5 days. Keywords: Incomplete, Infant, Kawasaki Disease [P-033] Septic arthritis in an 8 month-old,a challenging diagnosis Burcu Karadas1, Sule Nursah Ayata2, Nurcan Yusufoglu1, Zehra Esra Onal1, Cagatay Nuhoglu1 1 Department Of Pediatrics,Haydarpasa Numune Research and Training Hospital,İstanbul,Turkey 2 Department Of Family Medicine,Haydarpasa Numune Research and Training Hospital,İstanbul,Turkey Introduction: Septic arthritis is the invasion of a joint by an infectious agent. Septic arthritis in infancy is a challenging clinical problem,because signs and symptoms may be subtle and overlap with those found in other conditions. Case presentation: A 8 month-old boy presented to our institution with an inability to weight bear on affected limb.His hip joint was pulled by his older sister a month ago,at that time he was seen at emergency department, radiologic findings were normal.On his second presentation the diagnosis was clinched when he developed more definitive signs and symptoms, with fever and increased white blood cell count; which are considered independent variables that best distinguishes septic arthritis from transient synovitis.The earliest plain film radiographic findings were soft tissue swelling around the joint and a widened joint space from joint - 31 - effusion,which our case was diagnosed. Magnetic resonance imaging revealed septic arthritis of hip joint. He underwent surgery and 4 weeks of antibiotics administered intravenously.During his stay at our clinic, he diagnosed with varicella and also complicated with cellulitis of knee.The entire duration of stay was 4 weeks until normalization of the C-reactive protein level. We are happy to report a good recovery without any complications at his 12-month follow up. (iv, 30 mg/kg/day) for 3 days and 2mg/kg once daily per oral after pulse therapy. During the period of interruption that we reduced steroid doses, patient’s pulmonary hemorrhage attack repeated. Immunological tests were already normal and no signs of renal involvement for vasculitis. With all of these findings, 2mg/kg/day cyclophosphamide added to the therapy at follow-up. We planned to perform open lung biopsy when patient’s attack ended to check our diagnose. Conclusions: Septic arthritis of the hip is a true orthopedic emergency; delay in diagnosis or treatment may result in irreversible damage to the joint and disastrous complications in the pediatric patient,including complete destruction of the articular cartilage and the underlying epiphysis, loss of the adjacent growth plate, and dislocation of the joint. Conclusions: Idiopathic pulmonary hemosiderosis is a diagnose that exclude other alveolar diseases. Pulmonary hemorrhage can be seen with atipic systemic involvement and immunologic signs, expecially in childhood. It should be kept in mind that patient’s clinic and laboratory signs can get clear in the follow up period. Keywords: Pulmonary Hemorrhage, Anemia, Children With prompt treatment, all complications might be avoided, and normal function and future growth may be preserved. The keys to proper management are a high index of suspicion in any child with joint dysfunction and strict adherence to the principles for treatment. Keywords: septic arthritis, early diagnosis, treatment [P-034] Management of pulmonary hemorrhage in a pediatric patient: Case report Coşkun Armağan1, Seda Şirin Köse2, Suna Asilsoy2, Nevin Uzuner2 1 Department of Pediatrics, Dokuz Eylul University, Izmir, Turkey 2 Department of Pediatric Allergy & Immunology, Dokuz Eylul University, Izmir, Turkey Background&Aims: We aimed to raise awareness to management of pulmonary hemorrhage and vasculitis in pediatric patients. Methods: This is a case report of pulmonary hemorrhage in a 13-year-old child. Introduction: Pulmonary hemorrhage is a clinical condition characterized by severe clinical signs which includes respiratory distress, hemorrhage into the alveolus, cough, hemoptysis and anemia. Bilateral diffuse alveolar radiopacities are seen on chest radiograph. Etiology is reviewed by the presence of alveolar capillaritis, promote lung biopsy and immunofluorescence staining. In this study, a 13-year old child diagnosed with pulmonary hemorrhage at our clinic was presented. Case: 13-year-old boy, referred to our hospital with anemia in blood count and respiratory distress findings, consistent with bilateral pulmonary hemorrhage was detected in tomography. Patient’s pulmonary function test was FEV1:55%, FVC:52%, FEV1/FVC:103%, PEF:96%, MEF25-75:59%. All of immunological markers was negative. We performed flexible bronchoscopy. Lung biopsy results was non-specific and there were hemosiderin-containing macrophages in bronchoalveolar lavage. With all these findings, idiopathic pulmonary hemosiderosis was thought as preliminary diagnose. We started to give pulse methylprednisolone therapy [P-035] A case of brucella admitting with bicytopenia and deep thrombocytopenia Deniz Aygun1, Duhan Hopurcuoglu2, Fatih Varol3, Turkay Rzayev2, Haluk Cokuras1 1 Division of Pediatric Infectious Diseases, Clinical Immunology and Allergy, Istanbul University Cerrahpasa Faculty of Medicine 2 Department of Pediatrics, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey 3 Department of Pediatric Emergency Medicine, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey Introduction: Brucellosis is a widespread and potentially life-threatening multisystem zoonotic disease caused by intracelluler, gram-negative, non spore-forming bacteria of the genus Brucella. Clinical signs and symptoms are quite variable because all the organs can be affected by the microorganism. Hematologic findings are also non-specific and return to normal with appropriate antimicrobial therapy. Herein, we present a case of brucellosis presenting with massive hepatosplenomegaly, bicytopenia and deep thrombocytopenia. Case: A previously healthy 10-year-old male patient was admitted with a two-month history of weakness, high fever, weight loss. He was a member of a family living in a village and he consumed unpasteurized milk and milk products. On physical examination he had impaired general condition with 38.60C body temperature, firm hepatomegaly of 6 cm and splenomegaly of 9 cm. Laboratory analysis revealed anemia (8.5 g/dl), leucopenia (2200/mm3) and thrombocytopenia (4000/ mm3). The results of blood chemistry were within normal limits. Peripheral blood smear and bone marrow revealed no blast. The tube agglutination test was positive with 1/320 titer for Brucella. Bone marrow culture and hemoculture yielded gram negative coccobacilli, as Brucella spp. Rifampicin, trimethoprim sulfamethoxazole and amikacin combination therapy was started. The patient is currently in the third week of treatment, he is asymptomatic and pancytopenia returned normal levels. Conclusion: Brucellosis is still a challenging worldwide health problem caused by ingestion of unpasteurized milk or undercooked meat from infected animals, or - 32 - close contact with their secretions, milk and excrement. Various degrees of hematological manifestations can be seen during the course of the disease. Brucella should be kept in mind in patients admitting with bicytopenia and pancytopenia especially for our country which has a high Brucella seroprevalence. Keywords: brucella, bicytopenia, thrombocytopenia [P-036] A case of embryonal rhabdomyosarcoma presenting as parotitis Deniz Aygun1, Başak Koç2, Emre Gök3, Haluk Cokugras1 1 Division of Pediatric Infectious Diseases, Clinical Immunology and Allergy, Istanbul University Cerrahpasa Faculty of Medicine, Istanbul, Turkey 2 Instution of Oncology, Istanbul University Cerrahpasa Faculty of Medicine, Istanbul, Turkey 3 Department of Pediatrics, Istanbul University Cerrahpasa Faculty of Medicine, Istanbul, Turkey Rhabdomyosarcoma(RMS) is the most common soft tissue neoplasm seen in childhood that usually arises in the head and neck region. Rhabdomyosarcoma is an uncommon malignant mesenchymal neoplasm, accounting for 5% of all childhood malignancies. Unfortunately, it is often misdiagnosed as common benign conditions. Here we describe a case of embryonal RMS presenting with swelling of parotis region and fever mimicking parotitis. A previously healthy 3 years old boy admitted with a 10 days history of fever, irrability, pain and left sided swelling of parotis region. Physical examination revealed a diffuse, tender, unilateral swelling of 6x5 cm in diameter in the left parotid region. It was hard and not fluctuated. The rest of the physical examination was unremarkable. A complete blood count revealed a total leukocyte count of 14600/mm3, Haemoglobin 10.5 gr/dl, platelet count as 359,000/mm3. Erythrocyte sedimentation rate was 39 mm/hour, CRP level was 2.5 mg/dl. Ultrasonography displayed soft tissue swelling with multilobulated septation in left parotid gland which measured 32x25 milimeters approximately. Concerning clinical symptoms, increased inflammatory markers and radiological findings he was diagnosed as parotitis and empirically, intravenous ampicillin-clindamycin combination was started. After four days of therapy, the swelling was not regressed. Because of the atypical clinical course and unresponsive to antibiotic treatment, computed tomography was performed which suggested malignancy. Biopsy was performed and the histopathological evaluation of the sample adjusted embryonal rhabdomyosarcoma. Rhabdomyosarcomas of the parotid gland are the most lethal forms of salivary gland malignancy in children. They have a rapid onset and usually present with advanced disease. So every swelling of parotis gland is not always parotitis and rhabdomyosarcoma should be kept in mind in patients unresponsive to antibiotics. Keywords: Rabdomyosarcoma, parotitis [P-037] A case of antenatal diagnosed hyperimmunoglobulin M syndrome Deniz Aygun1, Ayper Somer2, Zehra Yavas3, Mine Kucuk3, Nuran Salman2 1 Department of Pediatric Infectious Diseases, Clinical Immunology and Allergy, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey 2 Department of Pediatric Infectious Diseases and Clinical Immunology, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey 3 Department of Pediatrics, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey Hyper-IgM syndrome (HIGM) is a rare primary immunodeficiency disorder characterized by severe defects of both cellular and humoral immunity due to impaired expression of CD40 ligand on activated T lymphocytes. Affected patients typically have elevated or normal serum IgM and decreased Ig G levels. Herein, we report an antenatal diagnosed hyperIgM syndrome having a sibling of the same disease. A 2-year-old male patient admitted with gingival redness and hypertrophy. His brother was diagnosed as X linked hyper-IgM syndrome at 10 years old due to recurrent infections and chronic diarrhea. He was the son of nonconsanguineous parents and genetic analysis of the mother showed a heterozygote pattern, indicating carrier status. Our patient was also diagnosed antenatally as X linked hyper-IgM syndrome and gene analysis of the CD40L gene revealed GG to CC substitution of Try 140 in 440-441 position. But the parents refused medical abortus and did not come to hospital until two years old despite of history of recurrent infection. The rest of his physical examination was unremarkable. Laboratory analysis revealed bicytopenia and neutropenia. Bone marrow aspiration did not reveal malignancy. Flow cytometric analysis of T, B, and NK cells revealed normal values. Serum immunoglobulin G level was low, 139 mg/dl, IgA: 47 mg/dl and IgM: 147 mg/dl. Antenatal diagnosis of hyper-IgM syndrome was confirmed and he was placed on intravenous immunoglobulin. Hyper-IgM syndrome is a combined immunodeficiency disorder characterized by elevated or normal serum IgM and decreased IgG, IgA, and IgE due to defective immunoglobulin class switching. Identification of mutations in this rare disorder will help in genetic counselling in affected families which could be further useful in carrier detection and prenatal diagnosis. Keywords: hyperimmunoglobulin M, antenatal - 33 - [P-038] Ataxia-telangiectasia presenting with hyperimmunoglobulin M syndrome: report of two cases Deniz Aygun1, Ayper Somer2, Mine Kucuk3, Burak Tatlı4, Nuran Salman2 1 Department of Pediatric Infectious Diseases, Clinical Immunology and Allergy, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey 2 Department of Pediatric Infectious Diseases and Clinical Immunology, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey 3 Department of Pediatrics, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey 4 Department of Pediatric Neurology, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey Ataxia-telangiectasia is an immunodeficiency syndrome with a wide-spectrum of findings including progressive cerebellar ataxia, oculocutaneous telangiectasia, ionizing radiation hypersensitivity and recurrent sinopulmonary infections. Patients usually have low levels of IgG, IgA, and IgE. About 10% of patients show raised serum IgM concentrations during the course of the disease, but it is unusual to find a high level of IgM at onset of the disease. Herein, we report two cases of ataxia-telangiectasia presenting with hyperimmunoglobulin M syndrome. Case 1: A 2-year-old male patient admitted with ataxia and recurrent infections including pneumonia. He was the son of consanguineous parents and he had a brother diagnosed as ataxia-telangiectasia. Physical examination revealed bilateral ocular telangiectasia and ataxia. Serum immunoglobulin G level was 0 mg/dl, IgA: 2.5 mg/dl and IgM: 618 mg/dl. In lymphocyte subtypes CD3 was %42, CD4 %25, others were normal. Serum alfa feto protein was elevated. He was diagnosed as ataxia-telangiectasia with hyperimmunoglobulin M syndrome and started monthly intravenous immunoglobulin infusion. Case 2: A 3-year-old female patient admitted with neuromotor retardation and recurrent sinopulmonary infections. She was the son of consanguineous parents and had a cousin diagnosed as ataxia-telangiectasia. Physical examination revealed only ataxia, the rest of was unremarkable. Serum immunoglobulin G level was 148 mg/dl, IgA: 44 mg/dl and IgM: 414 mg/dl. The proportions of lymphocyte subtypes and serum alfa feto protein were normal. She was also diagnosed as ataxiatelangiectasia with hyperimmunoglobulin M syndrome and started monthly intravenous immunoglobulin infusion. Ataxia-telangiectasia and hyper-immunoglobulin M syndrome are both primary immunodeficiency diseases caused by different genetic defects. Ataxiatelangiectasia patients presenting with decreased serum immunoglobulin G and A and with normal or elevated immunoglobulin M levels are often misdiagnosed as hyperimmunoglobulin M syndrome. Keywords: Ataxia-telangiectasia, hyperimmunoglobulin M [P-039] Congenital lobar emphysema with glaucoma and inguinal hernia Deniz Aygun1, Gonca Tekant2, Yusuf Can Dogan3, Haluk Cokugras1, Yıldız Camcıoglu1 1 Department of Pediatric Infectious Diseases, Clinical Immunology and Allergy, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey 2 Department of Pediatric Surgery, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey 3 Department of Pediatrics, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey Congenital lobar emphysema (CLE) is a rare congenital abnormality that is characterized by air trapping and overdistension of one or more lobes of lung with subsequent compression of adjacent structures leading to an atelectasis of the remaining lung parenchyma and mediastinal displacement. CLE presents with recurrent cough and wheezing within six months of life. Herein, we report congenital lobar emphysema with glaucoma and inguinal hernia in a 1 year old boy because of rare assosiation. A 1 year old boy having glaucoma and inguinal hernia admitted with history of recurrent pulmonary infections, chronic cough. Physical examination revealed bilateral crackles and rhonchi at lower zones. The blood parametere were normal. Chest X-ray shows hyperlucent and hyperexpanded left lung with mediastinal shifting to the right side without any visible visceral pleural lining. Computed tomography (CT) scan of the thorax revealed an emphysematous left upper lobe with tracheal and mediastinal shift to the right side. Both chest X-ray and CT scan confirmed the congenital lobar emphysema. On the echocardiography, there was no evidence of congenital cardiac anomalies. The patient had a left posterolateral thoracotomy with left upper lobectomy by pediatric surgery department. He had an uneventful postoperative course and was discharged from the hospital within one week. Histopathological examination of the surgical specimen revealed an emphysematous parenchyma with peribronchiolar lymphocyte infiltration. Chest x-ray should be performed in patients having history of recurrent pulmonary infections to rule out congenital lobar emphysema. Cardiovascular abnormalities are the most common accompanying congenital anomalies to CLE. Renal, gastrointestinal, musculoskeletal system anomalies are reported but to our knowledge this is the only case in the litherature having glaucoma and inguinal hernia with CLE. Keywords: Congenital lobar emphysema, glaucoma, hernia - 34 - [P-040] A case of idiopathic hypereosinophilic syndrome in a two years old girl [P-041] Deniz Aygun1, Hande Kumkumoglu3, Turkay Rzayev2, Hilmi Apak3, Tiraje Celkan3, Haluk Cokugras1 1 Department of Pediatric Infectious Diseases, Clinical Immunology and Allergy, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey 2 Department of Pediatrics,Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey 3 Department of Pediatric Hematology and Oncology, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey Idiopathic hypereosinophilic syndrome(HES) is defined as the presence of prolonged and over-production of eosinophils (>1.5 × 109/L) without an identifiable underlying cause. The clinical manifestations range from asymptomatic eosinophilia to aggressive disease complicated by life-threatening end organ involvement. Herein, we report idiopathic hypereosinophilic syndrome in a two years old girl, because it is extraordinarily rare in children. A previously healthy 2 years old girl admitted with fever and abdominal pain. She did not have any history of asthma, allergic disease or any disease before. Her physical examination was completely normal. A complete blood count (CBC) showed significant eosinophilia (77%), with an abso¬lute esosinophil count of 49,100. The total leukocyte count was 63,600/ mm3, haemoglobin 10.8 gr/dl and platelet count 220,000/mm3. Erythrocyte sedimentation rate, CRP and laboratory analysis were normal. Serum immunoglobulin levels were normal. Food allergy panel was negative. The chest x-ray, echocardiography and ultrasonography were normal. Investigations for parasitic infections, including toxocariasis, enterobius vermicularis and feces analysis were all negative. Bone marrow was full of eosinophils, but otherwise, including immunophenotype and cytogenetic analysis, it was normal. Serum tryptase level was normal. Purified protein derivative skin test was negative. A diagnosis of the hypereosinophilic syndrome was made on the basis of the presence of hypereosinophilia with no other identifiable cause or organ involvement. Treatment with prednisolone was started in a dose of 1 mg/kg/day. Hypereosinophilic syndromes is a rare condition in the pediatric age-group with a wide range of clin¬ical manifestations. Hypereosinophilic syndromes is diagnosed after exclusion of underlying diseases known to cause and the natural course of the disease is usually self-limiting in children as in our patient. Keywords: hypereosinophilic syndrome, child, idiopathic The effect of phototherapy treatment in the neonatal period, on the development of asthma, allergic rhinitis and atopic dermatitis Mehtap Sak, Mahmut Doğru, Feyza Mediha Yıldız, Serpil Değirmenci, Özlem Temel, Özlem Erdede, Derya Gültekin Başboğa Zeynep Kamil Training and Research Hospital Background&Aims: Icterus is a visible symptom of indirect hyperbilirubinemia. When serum total bilirubin (STB) level exceeds 5-6 mg/dl, icterus becomes visible to eye. Within the first week of birth, icterus can be seen in 60-80% of newborns and more than 80% of premature babies. 10-15% of the patients with hyperbilirubinemia should be treated with phototherapy. Allergic diseases including asthma, allergic rhinitis and atopic dermatitis are frequently observed in childhood period and the frequency is increasing for the last 30 years, especially in developed countries. Genetic effects cannot be the only reason behind this increase, environmental factors should be considered as well. Methods: This study analyzes 621 children between the ages of 3 and 17, including a patient group of 371 who received phototherapy and a control group of 250 who did not receive phototherapy. ISAAC survey was applied to all cases to diagnose allergic diseases. For patients with allergic diseases, eosinophil and total IgE levels were analyzed and skin prick test was applied. Results: In 25.5% (87 patients) of patient group and 27.2% (68 patients) of control group diagnosed asthma and/or allergic rhinitis and/or eczema were observed. There was no meaningful statistical different between patient and control groups in terms of diagnosed asthma and/or allergic rhinitis and/or atopic dermatitis (p=0,290). Atopy distribution in patient and control group is respectively 50% (16 patient) and 57,8% (22 patients). In skin tests applied to patients with allergic diseases in patient and control groups sensitivity (atopy) diagnose did not show meaningful statistical difference (p=0.509). Conclusions: In this study, we analyzed the relationship between phototherapy and the frequency of childhood asthma, allergic rhinitis and atopic dermatitis diagnosis. As a result of this study, we did not find a meaningful relationship between phototherapy treatment and asthma, allergic rhinitis and atopic dermatitis. Keywords: phototherapy, asthma, atopic dermatitis - 35 - [P-042] [P-043] The general features of children and adolescents with Hashimoto’s Thyroiditis Alstrom Syndrome: A case report Didar Arslan, Heves Kırmızıbekmez, Feyza Mediha Yıldız, Özlem Temel, Özlem Erdede, Serpil Değirmenci, Derya Gültekin Başboğa Zeynep Kamil Training and Research Hospital Background&Aims: Hashimoto’s thyroiditis is the most common reason of goiter and acquired hypotyroidism in children and adolescents. It is an autoimmune disease intrinsic to thyroid tissue mediated by genetic and environmental factors. In this study, we aimed to review our patients’ data with Hashimoto’s thyroiditis and compare results with literature findings. Methods: In this study, patients data with Hashimoto’s thyroiditis, who were being followed up in Zeynep Kamil Training and Research Hospital, Pediatric Endocrinology Department were reviewed retrospectively. Results: A total of 114 patient were evaluated; 102 female (89,5%) and 12 male (10,5%), the ratio of female to male was 8,5/1.The average age of the patients at diagnosis was 11,91±3,26 years. Based on symptoms, 23 patient (20,7%) had swelling in neck,19 (17,1%) had increased weight, 5 (4,5%) had the complaints of short stature and growth retardation. 8 patient were tested (7%) because of accompanying Diabetes Mellitus, and 6 (5,3%) for a positive family history of Hashimoto’s thyroiditis, 10 (8,8%) patient were diagnosed through routine laboratory tests. In the examination of thyroid gland on the first admission; 55 (48,2%) patients have goiter. Laboratory evaluations revealed; 47 (41,2%) patients were euthyroid, 46 (40,4%) patients were subclinical hypothyroid, 10 (8,8%) patients were overt hypothyroid, 3 (2,6%) patients were subclinical hyperthyroid, 8 (7,0%) patients were overt hyperthyroid. Antithyroid peroxidase antibodies were positive in 109 (95,6%) of the patients and antithyroglobulin antibodies were positive in 105 (92,1%) of the patients. Nodule was found in 22 of patients determined by thyroid ultrasonography. Radiation exposure was statistically significant between groups: 7 (13,2%) patient under 12 years old, only 1 (1,9%) patient over the age of 12 years. Conclusions: The patients who have clinical and laboratory evidence for thyroid disease should be tested for autoimmune thyroiditis. Keywords: children, adolescents, Hashimoto’s Thyroiditis Didem Ocal Topcu, Gamze Ozgurhan, Bahar Caran, Bulent Hacıhamdioglu Suleymaniye Maternity and Children’s Training and Research Hospital, Department of Pediatrics, Istanbul, Turkey Introduction: Alstrom syndrome is a very rare autosomal recessively inherited disorder that can lead to infantile-onset of dilated cardiomyopathy, blindness, hearing impairment, obesity, severe insulin resistance, hepatic and renal dysfunction. We report a 14 year-old boy who diagnosed as Alstrom Syndrome. Case Presentation: A 14-year-old boy was admitted to pediatric endocrinology department due to obesity. He was the first child of consanguineous parents, and he was born with a normal birth weight. There was a history of transient heart failure due to dilated cardiomyopathy in infantile period. Also he has been followed by retinal dystrophy which was first detected in infantile period. He has been treated with levothyroxine and metformine because of hypothyroidism and insulin resistance respectively. Severe acanthosis nigricans was noted in the neck, axillary region and fingers (Figure 1) on physical examination. His testes were 15/15 ml with normal penile length on external genital examination. His anthropometric measures were as follows: weight 107,8 kg (>95th percentile), height 172 centimeters (50-75th percentile). His body mass index was 36.2 kg/m2 (>95th percentile).His blood pressure was measured as 140 mmHg/90 mmHg. He had been suffering from photophobia, decreased vision and hearing loss for a long time. Cone-rod dystrophy and sensorineural hearing loss was detected by fundoscopic examination and audiogram respectively. In laboratory investigations; serum fasting glucose 98 mg/dl, fasting insulin 285.2 mIU/ml, C-peptid 13.18 ng/ml, HbA1c: 7.8%, alanine aminotransferase 131 IU/l and aspartate aminotransferase 80 IU/l, creatinine 0.8 mg/dl. Patient had hypertriglyceridemia with low HDL cholesterol level (361 and 29 mg/dl respectively). He has been diagnosed as Alstrom syndrome clinically. Conclusions: There is a need of detailed medical history and physical examination by pediatricians to confirm diagnosis of rare syndromes. Alstrom Syndrome should be kept in mind by pediatricians in obese children with early onset severe insulin resistance and retinal dystrophy. Keywords: Alstrom syndrome, insulin resistance, retinal dystrophy - 36 - [P-044] [P-045] Dilara Ece Toprak1, Tanyel Zübarioğlu2, Ertuğrul Kıykım2, Mine Aslan3, Mehmet Şerif Cansever4, Ayşe Çiğdem Aktuğlu Zeybek2 1 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatrics, Istanbul, TURKEY 2 Istanbul University Cerrahpasa Medical Faculty, Department of Pediatrics Division of Nutrition and Metabolism, Istanbul, TURKEY 3 Istanbul University Cerrahpasa Medical Faculty, Department of Radiology, Istanbul, TURKEY 4 Istanbul University, Cerrahpasa Medical Faculty Central Laboratory, Istanbul, TURKEY Wolman disease is caused by the complete loss of lysosomal acid lipase (LAL) activity which is essential for hydrolysis of cholesterol esters and triglyserides. It presents with vomiting, diarrhea, poor weight gain and hepatomegaly subsequently leading to death in early infancy. Diagnosis is based on the evaluation of enzymatic activity for LAL and LIPA gene sequencing. Here, an 18 days old female patient who was diagnosed as Wolman disease is reported. Diagnosis was confirmed with LIPA gene sequencing by showing novel mutation that was not reported before. An 18 days old female patient was admitted to our department for the etiological evaluation of hepatosplenomegaly and elevated liver enzymes. The first two children of the family who had similar complaints died in early infancy. Her physical examination revealed mild coarse facial features, mild macroglossia and hepatosplenomegaly. In her initial laboratory investigations, elevated plasma triglyceride and total cholesterol levels accompanied by remarkably reduced plasma high density lipoprotein level was found. Her liver enzymes were moderately elevated and she had a mild hypoalbuminemia. Abdominal sonography revealed hepatosplenomegaly and surrenal calcification bilaterally. Magnetic resonance imaging of the abdomen demonstrated significant enlargement of liver and spleen, as well as the surrenal glands. Having a family history of two siblings’ death, elevated liver enzymes and dyslipidemia in our patient and radiological findings led us to consider LAL deficiency in differential diagnosis. Decreased LAL activity was detected in dried blood sample. LIPA gene sequence was performed to confirm the diagnosis, and she was found in homozygote state for novel mutation that has not been previously described in the literature before. Wolman disease is a rare cause of hepatic failure that causes death in early infancy. Diagnosis should be considered in the existence of dyslipidemia and hypertransaminasemia. Diagnostic confirmation should be performed for genetic counseling. Keywords: Wolman Disease, LAL deficiency, genetic mutation Dilara Fucucuoglu, Ozlem Bostan Gayret, Meltem Erol, Ozgul Yigit, Emine Erdogan Kutanis, Ahmet Sener Bagcilar Training and Research Hospital, Pediatrics, Istanbul, Turkey Background&Aims: Recurrent abdominal pain is a common complaint in childhood. Abdominal epilepsy (AE) is a very rare cause of this clinical entity which is characterized by episodic paroxysmal abdominal and periumbilical pain, various abdominal complaints, specific abnormalities on electroencephalography (EEG) and good response to antiepileptic drugs. Wolman Disease In A Turkish Infant: A Novel Mutation Of Lysosomal Acid Lipase Gene An Uncommon Cause of Abdominal Pain: Abdominal Epilepsy Case: In this report, we present the case of a 12-year-old girl diagnosed with AE who was admitted to the emergency department because of recurrent periumblical abdominal pain, vomiting and dysuria. She had been admitted to the hospital several times in the last month. Her medical history did not include seizures or trauma. She had been followed up by pediatric nephrology department for having bilaterally grade 4 vesicoureteral reflux. One year ago she had bilaterally Cohen operation. She was hospitalized several times for urinary tract infections and uses drugs for hypertension, antibiotics for prophylaxia. On physical examination, the patient’s weight was 43 kg (50 p), and her height was 147 cm (50 p). System examinations were normal. Routine biochemical tests, acute phase reactants, hemogram, complete urinalysis and blood gas analysis were normal. There were no parasites or occult blood on stool examination. Urine and stool cultures were negative. Familial Mediterranean fever (FMF) gene mutation analysis and serum amiloid-A level were normal. The patient was assessed in the pediatric surgery department using abdominal USG and supine and erect abdominal graphy; however, pathology requiring surgical intervention was not detected. Electroencephalography revealed epileptiform activity characterized by sharp and slow wave complexes. Valproic acid was started. After treatment with the drug, the abdominal pain attacks did not recur. Conclusions: After exclusion of known causes, diagnosis of AE should be considered in children with episodes of paroxysmal abdominal pain. Anticonvulsant therapy should be initiated after a diagnosis of AE. Keywords: Abdominal epilepsy, recurrent abdominal pain, childhood [P-046] Spontaneous Pneumomediastinum in an 4-Year-Old Girl Okan Yüce, Dilara Fucucuoglu, Ozgül Yiğit, Meltem Erol, Zübeyde Kilinc Yaprak Bagcilar Treaning and Research Hospital, Istanbul, Turkey Background&Aims: Pneumomediastinum is an uncommon entity in pediatric practice. It is defined as free air or gas contained within the mediastinum, which - 37 - almost invariably originates from the alveolar space or the conducting airways. It is rarely symptomatic and diagnosed coincidentally. Clinical appearance spreads from asymptomatic to the life-threatening respiratory insufficiency. Spontaneous pneumomediastinum in children mostly caused by elevated intrathoracic pressure, but malnutrition can decrease the quality of the alveolar walls. Thoracic pain and dyspnea are the most common symptoms. Pneumomediastinum is diagnosed by posterior-anterior and lateral chest X-ray radiologically. In this article we present a 4 years old girl with spontaneous pneumomediastinum. Case: Our patient presented to emergency room with retrosternal chest pain. Her personal and family medical history revealed no significant findings. She wasn’t injured. Body weight was 13.4 kg(3-10 p). Her vital signs were normal: hemoglobin oxygen saturation 99% in room air, heart rate 80 beats/min, respirations rate 28/min, blood pressure 88/63 mm Hg and body temperature 36.8°C. Electrocardiography was normal. Physical examination revealed no visible haematoma, echymosis, redness or swelling on the chest wall. There was no palpable tenderness or subcutaneous emphysema over the neck, chest, face or shoulders. There weren’t any external signs of sternal and rib contusions or fractures. On the chest radiogram there was a suspect thin band of gas revealed between the heart and diaphragm. Computed tomograpyh of the chest revealed the presence of air in the parabronchial and perivascular connective tissue and around the anterior to the heart. We consigned the patient to a university hospital to the pediatric surgery department. Conclusions: In this article we want to emphasize that pneumomediastinum should be considered in children with chest pain without distinct clinical signs and symptoms. Keywords: Spontaneous Pneumomediastinum, Chest pain, childhood [P-047] Vitamin D deficiency’s effect on the severity of bronchiolitis Huseyin Kaya, Ozgul Yigit, Meltem Erol, Ozlem Bostan Gayret, Dilara Fucucuoglu, Can Polat Bagcilar Treaning and Research Hospital, Pediatric Department, Istanbul, Turkey Background&Aims: Vitamin D deficiency is frequent in children. Vitamin D takes an important role in bone metabolism, it also takes place in inflammatory conditions and in the course of infections. Bronchiolitis is a common infection in children younger than 2 years old. The aim of this study is to try to determine the relationship between vitamin D deficiency and the severity of bronchiolitis. Methods: We questioned in children younger than 2 years old who presented to the children emergency department in Bagcilar Training and Research hospital, the bronchiolitis frequency and the usage of the Vitamin D. Blood samples were taken from all children to measure the serum vitamin D levels. We separated the children in two groups according to the severity of the bronchiolitis: mild and severe. In classification we used the data hemoglobin oxygen saturation in room air, heart rate per minute, respirations rate per minute, having apnea, cyanosis and retractions. It has been classified statistically. Results: In our study there were 112 children between the ages 6,1 ± 4,9 months (median: 5 month). 47 children were female (%42) and 65 were male (% 58). The levels of vitamin D were significantly lower in severe group than the mild one. In the hospitalized group the levels were significantly lower than the outpatient group. The level of Vitamin D did not differ significantly the number of bronchiolitis attack. Conclusions: In our study we found that vitamin D takes an important role in the severity of bronchiolitis. If we can avoid the children from the vitamin D deficiency, we believe that we can reduce the duration of hospitalization. Keywords: Vitamin D deficiency, bronchiolitis, chilhood [P-048] Vitamin B12 Deficiency in infancy Gulsum Karakus, Ozgul Yigit, Ozlem Bostan Gayret, Meltem Erol, Dilara Fucucuoglu, Esra Ozcan Bagcilar Treaning and Research Hospital, Pediatric Department, Istanbul, Turkey Background&Aims: Vitamin B12 defiency is most ımportant cause of megabalastıc anemia. In this report, we presented two infant that exclusively breastfed by their mothers who consumed less animal protein. Case 1: Twelve month old male infant who exclusively breastfed, was admitted to hospıtal with paleness, vomiting, irritability, failure to gain weight, growth retardation, cough and breathing difficulty. His body weight and height were under 3 percentage. He was letharjic, hypotonic, he had hyperpigmention of palms ve knuckles. His lung finding has compatible with pneumonia and he has hepatomegaly. His hemoglobin was 5,9g/ dL, hematocrit 18%, MCV 90fl, plt: 41000 /mm3, the neutrophils were seen as hypersegmented. Iron and ferritin levels, biochemical profile and urine test results were normal, with normal blood and urine aminoacidography and plasma homocysteine level. Serum vitamin B12 level was 76pg/mL and serum folate level was 12,3ng/m. Case 2: Eleven month old female infant who exclusively breastfed was admitted paleness, growth retardation and failure to gain weight. She was irritable, letharjic, hypotonic. Her body weight and height were 10 percentage. Her hemoglobin was 5,8g/dL, hematocrit 17%, MCV 113 fl, plt: 118 000 /mm3, the neutrophils were seen as hypersegmented. Iron and ferritin levels, biochemical profile and urine test results were normal, with normal blood and urine aminoacidography and plasma homocysteine level. Serum vitamin B12 level was 30pg/mL and serum folate level was 15,6ng/m. The patients mother’s vitamin B12 level were low. The patients were given intramusculer cobalamin (200 μg/day for first week). After the start of therapy, their hemoglobin levels are rapidly improved, and a clinical improvement was observed after a few weeks. - 38 - Conclusions: During infancy, vitamin B12 deficiency may cause serious hematologic and neurological problems. The treatment of vitamin B12 deficiency is very crucial to prevent permanent neurological damage. Keywords: Vitamin B12, infancy, neurological deficit [P-049] Milroy Disease in a newborn Can Polat, Meltem Erol, Ozlem Bostan Gayret, Sahin Hamilcıkan, Ozgul Yigit, Dilara Fucucuoglu Bagcilar Treaning and Research Hospital, Pediatric Department, Istanbul, Turkey Background&Aims: Milroy disease is characterized by lower-limb lymphedema. Milroy disease, also known as primary congenital herediter lymphedema, is a genetic disorder. FLT4 (VEGFR3) is the only gene known to be associated with Milroy disease. Milroy disease is the rarest form of inhareted lymphedema. The disease is inhareted in an autosomal dominant manner. The most prominent feature at birth of Milroy’s disease is swelling in the feet. In this report we presented a newborn who diagnosed Milroy disease whose mother was also diagnosed with the same disease. Case: Our patient was given birth in an unknown gestastional week from an unfollowed syrian mother. His birth weight was 4140. He was admietted to the newborn intensive care unit. On physical exemination he had distinct lower-limb lymphedema and a systolic 1/6 degree murmur. Routine biochemical tests, acute phase reactants, hemogram, complete urinalysis and blood gas analysis were normal. There was no pathology in abdominal ultrasound graphy. On the transfontanel ultrasound graphy there were two cyctic lesions in the caudothalamic channels. The left one’s size was 3,5*2 mm, the right ones 4,1*2 mm. In the echocardiography we determined an atrioseptal defect. A lymphosintigraphy was planned. We referated the patient to the medical genetic department Conclusions: Milroy disease is a rare condition in pediatric clinics. We want to remember this rare disease because of this case with lower-limb lymphedema Keywords: milroy, lower-limb lymphedema, newborn [P-050] Two Patients With 22q11 Deletion Syndrome Presenting With Short Stature And Palatal Insufficiency Dilem Eriş, Gamze Özgürhan, Vildan Küçüksümbül, Rabia Nur Temiz, Bülent Hacıhamdioğlu Suleymaniye Maternity and Children’s Training and Research Hospital, Department of Pediatrics, Istanbul 22q11 Deletion Syndrome (22q11DS, DiGeorge Syndrome) presents with a very wide phenotypic spectrum, including the following: facial dysmorphisms; cardiac defects; velopharyngeal insufficiency; thymic hypoplasia; parathyroid hypoplasia; developmental delay. Growth deficiencies are common in patients, particularly during infancy and early childhood. Here, we report two children who diagnosed as 22q11DS while have been evaluated by short stature. Both patients were evaluated with short stature. Patient 1 was a 12-year-old girl and there was a history of palatal insufficiency (hypernasal speech and nasal regurgitation), which was occurred after adenoidectomy when she was eight years old. She was followed with left renal agenesis and atrioventricular septal defect. On physical examination, her height was 132.5 cm height (-3.7 SDS) she had mild facial dysmorphism (Figure-1), goiter, cubitis valgus, abdominal adiposity and rhinolalia. Patient 2 was a 7-year-old girl, and there was history of cleft palate surgery and adenoidectomy. She had medical history of frequent upper respiratory infections, speech difficulty and nasal regurgitation. On physical examination, her height was 113 cm height (-1.81 SDS) she had mild facial dysmorphism (Figure-2) and rhinolalia. Both patients had palatal insufficiency, mild facial dysmorphism and frequent upper respiratory infections; therefore, FISH screening had be planned for 22q11.2 deletion. FISH analysis revealed chromosome 22q11.2 deletion both patients. The decision to test for 22q11DS is easy if multiple defects and/or symptoms associated with the syndrome are present simultaneously. However, physicians should note that the signs and symptoms might be subtle. FISH screening should be planned for 22q11DS in every child with symptoms associated with velopharyngeal insufficiency. Short stature is associated with a number of syndromes, there is need detailed medical history and carefully physical examination for correct diagnosis of these syndromes. Keywords: 22q11, Palatal Insufficiency, Short Stature [P-051] A Girl Newborn with Male Phenotype Diagnosed as Congenital Adrenal Hyperplasia Dr. Mahmut Can Kızıl1, Prof.Dr. Birgül Kırel2, Dr. Tuğba Barsan Kaya3, Dr. Meliha Demiral2, Doç.Dr.Özge Aydemir3, Yrd.Doç.Dr.Hüseyin Aslan4 1 Pediatrics and Child Health, Osmangazi University Medical Faculty, Eskişehir, Turkey 2 Pediatric Endocrinology Department, Osmangazi Unıversity Medical Faculty, Eskişehir,Turkey 3 Neonatalogy Department, Osmangazi University Medical Faculty, Eskişehir, Turkey 4 Department of Medical Genetics, Osmangazi Unıversity Medical Faculty, Eskişehir, Turkey In childhood, congenital adrenal hyperplasia is the most common cause of adrenal insufficiency. It is an autosomal recessive inherited disease that is characterized with enzyme deficiencies in steroidogenesis. 21-hydroxylase deficiency with salt losing form is the most common type. CASE REPORT: Three weeks old infant was referred to our hospital for deteoriating in clinical condition with hyponatremia and hyperpotassemia after one week follow up in another hospital.He was born to a 23 years - 39 - old mother at 38th gestational weeks and did not need resuscitation. On physical examination body weight 2800 g, height 51 cm.He showed 2nd degree dehydratation symptoms, his genital examination was compatible with male sexuality, uretral orifice was in normal localization (compatible with prader stage 5 )but his gonads were not palpable.Laboratory evaluation revealed sodium:112 mEq/l, potassium:7,9 mEq/l,glucose:87 g/l, 17(OH) progesteron:92 ng/ml, DHEA-SO4:113,4 ug/dl, testosteron:70,7 ngdl, androstenedion:22,03 ng/ml. He was treated for adrenal crisis and congenital adrenal hyperplasia –salt wasting type. Karyotype analyse was resulted as XX and SRY gene research was resulted as negative.Genetic analysis for 21-hydroxylase deficiency was resulted as I2 splice and Del 8bp E3 homozygot mutations positive. Council consisting of geneticist, pediatric endocrinologist, neonatologist and pediatric surgeon concluded that feminizing corrective surgery is required. DISCUSSION: Female newborns with complete deficiency of 21-hydroxylase may have full developed male genitalia and if unrecognized, they are growth up as boys.Careful examination reveals undescended gonads which can be a clue for congenital adrenal hyperplasia. Early diagnosis, treatment and surgery can prevent adrenal crisis in this patients, it can also prevent families’ social and pshycological problems.So congenital adrenal hyperplasia is one of the most important disease should be implemented in national disease screening program in Turkey because of the high rates of intermarriage. Keywords: congenital adrenal hyperplasia, 21-hydroxylase deficiency, adrenal crisis [P-052] A Case with Fever and Rash Later Diagnosed as Hemophagocytic Syndrome Durdugül Ayyıldız Emecen1, Sermin Özcan1, Gülperi Yağar Keskin1, Dildar Bahar Genç1, Zeynep Yıldız Yıldırmak1, Gülşen Köse1, Ahmet Naci Emecen2 1 Şişli Hamidiye Etfal Education and Research Hospital, Pediatric Clinics, Istanbul, Turkey 2 Istanbul Medeniyet University Göztepe Education and Research Hospital Infectious Disease Department, Istanbul, Turkey Background&Aims: Hemophagocytic syndrome (hemophagocytic lymphohistiocytosis, HLH) is a potentially fatal phenomenon characterized by the activation of macrophages and T lymphocytes, dysfunction of cytotoxic T lymphocytes and naturel killer cells, overproduction of cytokines and hemophagocytosis leads to fever, hepatosplenomegaly and pancytopenia. HLH is divided into familial and secondary subtypes. With this case we aim that HLH should be kept in mind with febrile children despite treatment. Case: 3.5 months aged girl admitted to hospital with fever, millimetric maculopapular rash in scalp and body for two days. Parents were not relative. Respiratory, cardiac and neuromotor examinations were normal. Liver was 4 cm and spleen was 3 cm palpable. Hb: 9.9 g/dl, WBC:16000/ mm3, Neu:3040/mm3, Le:10570/mm3, Plt:18000/mm3, CRP:20 mg/L. No atypic cells in peripheral blood smear. Ceftriaxone and 1 gr/kg IVIG started with the diagnosis of sepsis. Fever, notropenia and trombocytopenia continued at the third day of treatment and the second dose of IVIG applied. No atypical cells were observed in bone marrow aspiration. Lymphocyte predominance and one macrophage with phagocytosed erythrocytes were seen; hypertriglyceridemia, hyperferritinemia, hypofibrinogenemia detected and diagnosis of HLH was made. Perforin mutation, TORCH panel, EBV and Parvovirus tests were negative. CMV IgM was positive but CMV PCR was negative. Immunoglobulines and lymphocytes subtypes were normal. Fever and pancytopenia persisted and dexamethasone 10 mg/kg started. Bone marrow aspiration was repeated on the 15th day of dexamethasone to indicate the response to the treatment and again phagocytosed erythrocytes were seen. The patient received third dose of IVIG but hepatosplenomegaly didn’t resolved. Neutrophil count was >1000/mm3 but no significant improvement in platelet and hemoglobin count observed so HLH 2004 protocol and bone marrow transplantation planned. Results: Because of HLH is a rare but fatal disease; it must be considered in patients with prolonged fever, hepatomegaly and pancytopenia. Early initition of treatment may be life-saving. Keywords: hemophagocytic syndrome, persistent fever [P-053] A thanatophoric dysplasia type I case with the mutation of p.R248C on FGFR3 gene Ebru Atas Aslan1, Hamdi Ograg2, Suzan Sahin2, Abdullah Barıs Akcan2, Huriye Nursel Elcioglu3, Munevver Kaynak Turkmen2 1 Department of Pediatrics, Faculty of Medicine, Adnan Menderes University, Aydın, Turkey 2 Division of Neonatology, Department of Pediatrics, Faculty of Medicine, Adnan Menderes University, Aydın, Turkey 3 Division of Genetics, Department of Pediatrics, Faculty of Medicine, Marmara University, Istanbul, Turkey Introduction: Thanatophoric dysplasia (TD) represents the most common lethal skeletal dysplasia (1). Molecular genetic analysis of the fibroblast growth factor receptor 3 (FGFR3) gene is useful for the prenatal diagnosis of TD, because FGFR3 is the only gene known to cause TD (2, 3). Here, a TD case with suspicion of the disease in prenatal ultrasonography, born with the dysmorphic features and confirmed diagnosis with molecular analysis is presented. Case: A baby boy, weighted 2985 grams, born at the 37th gestational week by cesarean section with APGAR scores of 7/8 as the first baby of the family, was hospitalized due to respiratory distress and dysmorphic features. Amniosynthesis performed due to the shortness of the longbones on ultrasonography during the second trimester revealed constitutional karyotype. On physical examination, the length of the baby was 39 cm (<10p), weight 2985 g (50-75p) and head circumference 39 cm (>90p). The baby had hypotonia, short neck, depressed nasal root, frontal bossing, edema of the scalp and - 40 - marked shortening of the longbones. He was intubated due to respiratory insufficiency and died on the 162nd day of his life. TD type I diagnosis was confirmed by molecular analysis of FGFR3 gene which showed heterozygote p.R248C mutation. Discussion: TD, as could be imagined from its name, is a very severe congenital anomaly and most of the cases are either stillbirth or lost during the first several hours of their life due to respiratory insufficiency. The rear only few case reports in the literature regarding to TD cases who could survive beyond the childhood period (4). Molecular diagnosis can relieve parental anxiety in such a low risk couple. This case is presented due to the relative long life span of a baby with TD and whose diagnosis could be confirmed by molecular analysis during his hospital stay. Keywords: thanataphoric dysplasia, FGFR3, genetic counselling [P-056] [P-054] Macrophage activation syndrome secondary to systemic juvenile idiopathic arthritis Elif Arslan, Soner Sazak, Saniye Berna Hamilçıkan, Emine Türkkan, Yelda Türkmenoğlu, Muhammet Nurullah Yakut, Ozan Özkaya Okmeydanı Training and Research Hospital, Department of Paediatric, Istanbul Macrophage activation syndrome (MAS) is caused by the excessive activation and uncontrolled proliferation of T-cells and well-differantiated macrophages. MAS has been reported in patients with almost any rheumatic diseases and most strongly associated with Systemic Juvenile Idiopathic Arthritis (SJIA). MAS can be dignosed by the presence of 5 of the following: fever, splenomegaly, cytopenia of 2 cell lines, hypertriglyceridemia or hypofibrinogenemia, hyperferritinemia, elevated soluble CD 25 (interleukin-2 receptor), reduced or absent NK cells, and bone marrow, CSF, or lymph node evidence ofhemophagocytosis. A case of 7 year old boy is presented here who refered to hospital with arthritis and fever. After hospitalization hyperferritinemia and hemophagocytosis was detected by the biopsy of bone marrow. With immunotherapy the patient induced remission. Keywords: macrophage activation, children, juvenile idiopathic arthritis [P-055] Coagulation Disorder And Direct Hyperbilirubinemia In A Newborn With Galactosemia disorder. Classical galactosemia appears due to deficiency of galactose-1-P uridyl transferase (GALT) which is essential for galactose metabolism pathway. Signs of disease begins with taking galactose rich nutrition as breast milk, cow milk or formulas containing lactose. Usually the disease begins after the first week of life with jaundice, vomiting, hypoglycaemia, cataract, nutrition problems, irritability, convulsion, weight loss, sepsis or liver failure. Restriction of galactose intake is lifesaving. In this case an eight days old infant brought to hospital with jaundice and weight loss is presented. In labaratory studies, baby had direct hyperbilirubinemia and prolonged coagulation parameters. With suspicion of galactosemia breast milk had been stopped and started lactose-free formula. In a few days bilirubin and coagulation parameters turned to normal. GALT level of baby was detected so low. Keywords: galactosemia, GALT, lactose free formula The Correlation Between Aortic Intima Media Thickness, Blood Lipid Profiles and Leptin Levels in Newborns with Intrauterin Growth Retardation Emel Akıncı Ataoğlu1, Vasfi Onur Vermezoğlu1, Öznur Vermezoğlu1, Derya Büyükkayhan2, Murat Elevli1 1 Istanbul Haseki Training and Research Hospital, Department of Pediatrics 2 Istanbul Medeniyet University, Department of Pediatrics Background&Aims: In this study,it was aimed to determine the aortic intima-media thickness (aIMT) as an indicator of preclinic atherosclerosis and its relationship betweenblood lipid profiles and leptin levels in newborns with intrauterin growth retardation (IUGR) hospitalised in NICU of Haseki Research and Education Hospital. We also aimed to to adress which children are prone to atherosclerosis and which mechanisms are responsible for this. Methods: 30 babies with IUGR and 30 babies with appropriate growth for gestational age (AGA) were included in the study.The demographic characteristics, biochemical values, arteriel blood pressures, weights and CRP levels were recorded. Aortic intima-media thickness and its relation with lipid profiles and leptin levels were also determined. The results were compared between groups. Results: The lipid profiles, abdominal aortic intimamedia thicknesses and leptin levels of IUGR babies were not signifacantly different than that of AGA babies Elif Arslan, Mustafa Kemal Özdemir, Evrim Çeliker Yapıcı, Berna Gümüşoğlu Akşahin, Avidan Kızılelma Yiğit, Yeşim Acar, Ömer Faruk Beşer, Bekir Yiğit Develi, Ozan Özkaya İstanbul Okmeydanı Eğitim ve Araştırma Hastanesi Çocuk Sağlığı ve Hastalıkları Galactosemia is a rarely seen carbonhydrate metabolism Conclusions: According to these results, IUGR babies are not more likely to develop atherosclerosis compared to AGA babies. Keywords: lipid profile, intima media thickness - 41 - [P-057] The Correlation Between Aortic Intima Media Thickness in Different Gestational Age Newborns Emel Akıncı Ataoğlu1, Öznur Vermezoğlu1, Vasfi Onur Vermezoğlu1, Derya Büyükkayhan2, Murat Elevli1 1 İstanbul Haseki Training and Research Hospital 2 İstanbul Medeniyet University Background&Aims: In this study,it was aimed to determine the aortic intima-media thickness (aIMT) as an indicator of preclinic atherosclerosis and its relationship between blood lipid profiles and gestational age in newborns those hospitalised in NICU of Haseki Research and Education Hospital. We also aimed to to adress which children are prone to atherosclerosis and which mechanisms are responsible for this. Methods: 60 babies have different gestational age (AGA) were included in the study. The demographic characteristics, biochemical values, arteriel blood pressures, weights and CRP levels were recorded. Aortic intima-media thickness and its relation with lipid profiles and gestational age were also determined. The results were compared between groups. Results: The lipid profiles and abdominal aortic intimamedia thicknesses were not significantly different in different gestational age groups. Conclusions: According to these results, Premature babies are not more likely to develop atherosclerosis compared to term babies. Keywords: intima media thickness, gestational age [P-058] A retrospective evaluation of causes of morbidity, and mortality among babies delivered in the Haseki Training and Research Hospital within the 3 years Ali Alkan1, Emel Akıncı Ataoğlu1, Derya Buyukkayhan2, Murat Elevli1 1 İstanbul Haseki Training and Research Hospital 2 İstanbul Medeniyet University Background&Aims: Our aim in this study is to evaluate morbidity, and mortality rates of the babies delivered between January 2009, and December 2011 so as to shed light on prospective planning studies, and relevant applications to be performed. Methods: We compared mortality, and morbidity data of the babies delivered in newborn registry between January 2009, and December 2011, and analyzed epidemiological data of these babies delivered in our hospital at gestational age of > 22 weeks with birth weights below 500 gr. 0.61, and 1.65 %, respectively. Causes of perinatal death were stillbirths (68.1 %), and congenital malformations (11.3 %), prematurity-related factors, and other etiologies in order of decreasing frequency. The most frequent indications for hospitalizations were neonatal hyperbilirubinemia (35.3 %), and suspect sepsis (13.4 %). Our median mortality rate among babies delivered before 32. gestational weeks within indicated three years was 14.5 percent. Conclusions: Providing adequate antenatal, and postnatal care for pregnants, and their newborns, and realization of deliveries under optimal conditions will dramatically decrease neonatal mortality rates. Keywords: Neonatal, mortality, morbidity [P-059] Congenital Syphlis Case Presented with Purulent Rhinorea and Skin Findings Emel Akıncı Ataoğlu1, Özlem Kosvalı1, Derya Buyukkayhan2, Murat Elevli1 1 İstanbul Haseki Training and Research Hospital 2 İstanbul Medeniyet University Congenital syphilia is a serious condition that transmit from mother to baby through transplasentally in all stages of pregnancy. The various clinical presentations depend on the gestational age of fetus, whether the mother was diagnosed or not, treatment of mother and immunologic response of fetus. Half of the syphilitic pregnancies could be resulted with spontaneous abortus, perinatal death and premature birth. The infection commonly transmit by intrauterine way, however ocaasionally the infection could transmit at the time of birth with genital contact. The two third of infected fetuses are asymptomatic at birth. Prematurity, low birth weight, elevated liver functions, hyperbilirubinemia, skin findings, thrombocytopenia, hemolitic anemia, lymphadenopathy, bone deformities, central nervous system abnormalities and fever are signs of symptomatic neonates. Our case was borned at 32 gestational age weighing 1930 gr. His mother had no control in her pregnancy and known to have condyloma. He was entubated and brought to neonatal intensive care unit. The lung X-ray of newborn showed the signs of respiratory distress syndrome and surfactan had been given. On follow up purulent rhinorrhea and maculopapular rashes occured. Laboratory findings showed pozitive tests for VDRL and TPHA. According to lomber punction he had no neurological involvement. On babygram there was no bone deformity. Congenital syphilis diagnosis was made and 10 days penicilin treatment was started. After two months hospital care, he was discharged with his controls were planned. Keywords: congenital, syphilis Results: 1905 live babies, and 32 stillbirths were delivered in the year 2011. During early neonatal period 12 babies exited. Rates of perinatal, and early neonatal mortality, and stillbirth were found to be 2.27, - 42 - [P-060] Other system examinations were normal. Diagnostic value of serum procalcitonin and C-reaction protein levels in neonatal sepsis Mehmet Ali Erceylan1, Emel Akıncı Ataoğlu1, Derya Büyükkayhan2, Murat Elevli1 1 İstanbul Haseki Training and Research Hospital 2 İstanbul Medeniyet University Early diagnosis of neonatal sepsis mortality reduction of morbidity is of great importance. Hemoculture growth factor is produced before the diagnosis of acute phase reactants are supported. Clinical applications, C-reactive protein (CRP) levels, monitoring is used for this purpose. In recent years, procalcitonin (PCT) has come into use in neonatal sepsis. PCT increase is earlier than CRP. In this study, neonatal sepsis diagnosis and follow-up CRP and PCT levels were compared. Study was conducted on 40 patients with the diagnosis of neonatal sepsis in the intensive care unit and 40 controls. Study group of the PCT and CRP levels before treatment were significantly higher than the control group (p <0.05). CRP and PCT levels before treatment, the treatment received after 72.hours compared CRP and PCT levels, showed a statistically significant decrease (p <0.05). PCT in sepsis as a result of early diagnosis and in monitoring response to therapy are used routinely to CRP were found to be useful. Keywords: Procalcitonin, neonatal sepsis [P-061] recurrent bacterial menengitis after traumatic head injury Emine Olcay Yasa, Asena Pınar Sefer, Ismet Dusmez, Doruk Gül Goztepe Research Hospital Istanbul/Turkey Introduction: Recurrent bacterial menengitis is defined as two or more episodes of meningitis separated by a period of convalescense and the complete resolution of all signs and symptoms and it is a rare condition which usually caused by head trauma, congenital anatomical defects,immunological deficiencies. An accurate diagnosis of underlying pathologly is crucial to prevent further episodes from occuring. In this case we highlighted the importance of investigation and diagnosis of underlying pathology which causes recurrent bacterial menegitis episodes. Case: 8 year old male patient applied to hospital by high fever,severe headache,nausia,vomiting and malaise. He took diagnosis of acute bacterial menengites by lumbar puncture and CSF examination. He was transported to our infectious disease deparment for internalitazion. [P-062] Varicella zoster case with ataxia and dyslexia Emine Olcay Yasa, Asena Pınar Sefer, Doruk Gül, İsmet Düşmez Goztepe Research Hospital Istanbul/Turkey Introduction: Acute cerebellitis is an inflamatory condition characterized by cerebellar disfunction. It’s generally bening condition however rarerly it may cause herniation and death. It may occur after some kind of infections and vaccinations.Infections such as varicella zoster,EBV,coxsacie,rubella,diphteria and pertusis; are the most common causes.Lead or cyanide poising, demiyelinating diseases and vasculitis may cause cerebellar inflamation. However;sometimes the etiology cant be detected.To diagnose idiopathic acute cerebellitis; other etiological factors should be excluded. In this case we describe varicella zoster cerebellitis who applied with vomiting, speaking and walking disability. Case: 5 years old male patient,6 days ago he was diagnosed as varicella zoster with the complain about multiple vesiculer skin eruptions.After 3 days he developed ataxia,dyslexia and severe nause -vomiting. He was internalized infectious dieases department. At admission, he was lethargic.Physical examination;he had generilazed multiple vesiculer skin eruption.ataxia +,romberg +, dysdiadokinesia +.He had no pathological reflexes, no meningial irritation finding.Other physical examination sings were normal. Hb:11.4 g/ dl,Plt:368000,Wbc:7.7,Neu:%42,Lenf:%49,Glucose:98 mg/dl,creatinin:0.46 mg/dl,Ast:30 u/lt,Alt:19 u/ lt,Na:141 meq/lt,K:4.5meq/lt,Cl:108 meq/lt,Ca:9.1mg/ dl,Crp:0.33 mg/dl Differential diagnosis; vzv cerebellitis,ADEM,mass lesion at posterior fossa of cranium,vasculitis,drug intoxication In order to exclude ADEM and posterior fossa tumor we took cranial MRI of the patient and the result was normal. He was diagnosed varicella zoster cerebellitis and 30 mg/kg/day acyclovir treatment was started. With the treatment;signs and symptoms of patient dramatically decreased. And the treatment was completed at 10 days.Then he was externated from hospital. Keywords: varicella zoster cerebellitis, ataxia, dyslexia Medical history;4 years ago he had traffic accident which cause traumatic head injury and he operated due to subdural hematoma. 1.5 year ago he had another acute bacterial meningites episode. At admission, he was lethargic,Gks:12,body temperature:38.5.Physical examination; neck stiffness +,Kerning+,dlr:+/+ ılr:+/+,had no patological reflexes.. Babinski -,motor exam of lower exst.:5/5 upper exst:5/5. - 43 - [P-063] [P-064] Emine Yurdakul Ertürk1, Özlem Özdemir2, İsmail Taşkıran3, Abdullah Erdil1 1 Department of Pediatrics, Medical Faculty, Ordu University, Ordu, Turkey 2 Department of Internal Medicine, Medical Faculty, Ordu University, Ordu, Turkey 3 Department of Gastroenterology, Medical Faculty, Ordu University, Ordu, Turkey Background&Aims: The upper gastrointestinal system (GIS) hemorrhages are the hemorrhage into the lümen from the upper oesophagus to the treitz ligament.It occurs in the childhood rarely then the adult ages.İn this case,we have presented an adolescent with the upper GIS hemorrhage after a one dose use of the paracetamol. Emine Yurdakul Ertürk1, Şükrü Küçüködük2, Canan Aygün2, Kemal Baysal3 1 Department of Pediatrics, Medical Faculty, Ordu University, Ordu, Turkey 2 Division of Neonatalogy, Medical Faculty, 19 Mayıs University, Samsun, Turkey 3 Division of Pediatric Cardiology, Medical Faculty, 19 Mayıs University, Samsun, Turkey Background&Aims: The transposition of the great arteries (TGA) which is a cyanotic cardiac abnormality constitutes approximately 5% of congenital cardiac disease. In this study, the investigation of some demographic and clinical characteristics of thirty patients who were diagnosed with neonatal TGA between 20062010 periods was aimed. İs the one dose of the paracetamol can be the cause of the gastrointestinal system hemorrhage? Transposition of the great arteries: Evaluation of the thirty neonatal patients The Case: The male,seventeen age adolescent who explain that he had any complaint before,has came to the emergency clinic with the handful hemorrhage after a nausea and vomiting.There was no characteristic in his brief and family history.We have learned that he took a one 500mg paracetamol tablet for the abdominal pain reason,five days ago.His appearance,vital parameters and physical examination were normal.Hb:14,6 gr/ dl, WBC:7000/mm3, platelet:259000/mm3, BUN:24 mg/dl, Cr:0,6 mg/dl, AST:17 U/L, ALT:12 U/L, Na:139 mmol/L, K:4,3 mmol/L, Cl:105 mm/L were determined. The coagulation parameters were normal.A nasogastric tube has been placed,the oral take has stopped,fluid replacement and antisecretuar treatment have started.180 cc bloody fluid from NG and melaena were determined,abdominal ultrasonography was normal. After the four hours of the treatment the hemorrhage was stopped and the hemoglobin level was stabil.The upper GIS endoscopy was done and a antral gastritis and a one bulber ulcer at 2-3 mm dimension (forrest3) were determined.İn the following,the patient had any complaint,had oral nutrition and he was discharged with the prescription and the advice for the polyclinic control. Conclusions: In many research,the paracetamol was detected as the reason for the upper GIS hemorrhage. Even the dosages age-appropriate or the using for the short term can be a reason to a hemorrhage.In our study the hemorrhage was associated with the one dose paracetamol take but we need the many similar studies for to be sure.On the other hand,the commun information must be done about the adverse effects of the paracetamol which is accepted safety for the stomach. Keywords: adolescent, hemorrhage, gastrointestinal system Methods: Within the 300 patients who were hospitalized in the Neonatal Intensive Care Unit of the Ondokuz Mayıs University with the diagnosis of congenital heart disease between January 2006 and December 2010, met the criteria to be included into the study and whose files were accessible, the files of the TGA detected patients in echocardiography (ECHO) were analyzed retrospectively. Results: The frequency of the transposition of the great arteries was determined as 10% (30/300). Nine of the cases (30%) were female and 21(70%) were male. The average diagnosis age of the patients was found as 9.53 days. While 16(53.4%) of patients were d-TGA, 14(46.6%) of them were isolated TGA and 26(86.6%) cases had additional cardiac disorder. In 30 cases, because of the cyanosis 16(53.3%) of the patients and because of the heart murmur 14(46.7%) of the patients were performed cardiological evaluation and ECHO. The arterial switch operation were performed on the 18(60%) of the cases. While the 14(46.7%) cases were discharging, 16(53.3%) of them died. Conclusions: The transposition of great arteries is the most common cyanotic congenital heart disease in newborns and infants. If the surgical interventions aren’t applied, the mortality rate is over 90% in the first year of life. Prenatal diagnosis of TGA is possible with a fetal ECHO. The fetal ECHO which will be applied in the prenatal period is extremely important for the prevention of cases that can be mortal due to complex congenital heart abnormalities and the prediction of surgical strategy which will be required after birth. Keywords: newborn, transposition of the great arteries - 44 - [P-065] [P-066] Emmanouela Sdona1, Dimitris Papamichail2, Takis Panagiotopoulos2, Pagona Lagiou3, Ariadne Malamitsi Puchner1 1 Department of Neonatology, Aretaieion Hospital, University of Athens Medical School, Athens, Greece 2 Department of Child Health, National School of Public Health, Athens, Greece 3 Department of Hygiene, Epidemiology and Medical Statistics, University of Athens Medical School, Athens, Greece Background&Aims: Term and late preterm neonates are considerably less prone to necrotizing enterocolitis (NEC), which is the most common gastrointestinal emergency in preterms. The aim of this study was to investigate a cluster of late preterm and term neonates (gestational age >= 34 weeks) with NEC symptomatology in a maternity hospital during a period of 14 months. Emrah Aydın Bahcelievler State Hospital Aim: To present diagnosis of desmoid tumor in a patient with recurrent upper abdominal pain and mass with normal ultrasound findings. Necrotizing enterocolitis affecting late preterm and term neonates Methods: Medical records of neonates with modified Bell stage >= IB NEC were reviewed, in addition to microbiological and environmental investigation. Variables collected included demographics, maternal/ delivery and neonatal factors, medications, procedures and feeding practices. Results: Out of 1841 late preterm and term neonates, 20 presented with NEC symptomatology at mean 4.6 days (2-8 days). Μean (± SD) birthweight was 2529.3 (493.04) grams and mean gestational age was 36.96 (1.48) weeks. Most (14/20) were born by caesarean delivery. Nearly all (19/20) resulted from high risk pregnancies (late preterm, twin, intrauterine growth restriction, in vitro fertilization, prolonged rupture of membranes, placenta praevia and maternal diseases – preeclampsia, gestational diabetes, thyroid disease, thrombophilia, autoimmune hepatitis, epilepsy, Turner syndrome and Hodgkin’s disease) and received postpartum intermediate care. No neonate had congenital cardiac disease and none required intubation, umbilical artery catheterization or blood transfusion. Apgar scores were equal or more than 71’ and 95’. All were formula fed (exclusively or partly). Ten neonates with suspected NEC (stage IB) had mild clinical course and were treated with antibiotics for less than 10 days with gradual resumption of feedings. Ten other neonates had stage >= II NEC, eight of whom underwent surgery, with no fatality. NEC lesions were mainly located in the colon. No pathogen could be identified. Conclusions: Late preterm and term neonates with predisposing clinical conditions, requiring postpartum intermediate care, are susceptible to NEC; feeding with breast milk seems to be the strongest preventive measure. Keywords: necrotizing enterocolitis, late preterm, term Preference of the Surgeon: The patient or the radiologist? Desmoid tumor Case: Fifteen-years-old boy had been operated due to acute appendicitis and intraabdominal abscess four years ago was admitted to our clinic with complaints of right upper quadrant pain and swelling for two years. Many ultrasound were performed but nothing was found. There was 6cm thickening and heterogeneity of right rectus abdominis muscle with a suspicious mass neighboring the liver at CT and MRI. He was operated and pathology was reported as desmoid tumor. Conclusion: Even basic studies doesn’t reveal any pathology if the patient’s complaint goes on surgeon must be aggressive for diagnosis in order not to miss out malignancies. Keywords: desmoid tumor, desmoid type fibromatosis, aggressive fibromatosis [P-067] One Month Ongoing Intussusception Emrah Aydın Bahcelievler State Hospital Aim: To present a case operated due to suspected intussusception and diagnosed as lymphoma Case: A six years old boy was admitted tour clinic with a complaint of colicky abdominal pain and bilious vomiting for the last month. No pathology was found at his examinations in other clinics. He had tenderness and defense at physical examination but no mass was palpated. All biochemical parameters were normal except leukocytosis. Abdominal X-ray was normal. 3cm long intussusception was found at ileocecal part at ultrasound. He had colicky abdominal pain at his follow up and bilious content at nasogastric tube. Pneumotic reduction was performed but no intussusception was found. Air passage to the proximal part of intestine was normal but there was a mass image at ileocecal valve. Edematous and pseudomembranous intestinal segment was found. There was multiple lymph nodes at mesenteric area. Ileoascendostomy was performed. Pathology was reported as Burkitt lymphoma. He received two cycles of chemotherapy at post-operative period. He is still event free for the last year. Conclusion: One must remember that lymphoma can be the lead point in intussusception. Keywords: intussusception, lymphoma - 45 - [P-068] Recurrent abdominal pain: Multiseptated gall bladder Emrah Aydın Bahcelievler State Hospital Aim: To present a patient was admitted to our clinic with recurrent abdominal pain and diagnosed as multiseptated gall bladder. Case: Seven years old boy admitted to our department with recurrent abdominal pain and diagnosed as multiple septation in gall bladder. All laboratory values were normal. There was no sludge in ultrasound. MRCP didn’t show any congenital abnormality. He has been abdominal pain only once in a time for the last year. Conclusion: Although a rare condition in childhood, patients with multiseptated gallbladder should be followed unless without clinical symptoms. Keywords: multiseptate gall bladder, abdominal pain [P-069] A rare cause of acute abdomen in prematurity: Intussusception Emrah Aydın Bahcelievler State Hospital Aim: To present a neonatal referred to our clinic due to acute abdomen and diagnosed as intussusception. Case: A newborn who didn’t have any prenatal problem born at 34th gestational weeks with normal spontaneous vaginal way, weighed 2500gr was monitored in intensive care unit for 10 days. Except being intubated for one day he was well on follow up and discharged to home. One week after discharge he was referred tour clinic with bilious vomiting and rectal bleeding. At physical examination he had normoactive bowel sounds and abdominal distension with tenderness on the right lower quadrant without defense or rebounds. White blood cell count was 22000/mm3 besides other values were normal. There was no free air under the diaphragm at abdominal radiography but abdominal ultrasound revealed 2cm ileocolic intussusception in the right lower quadrant. Operation decision were taken and ileocolic intussusception was revealed. Manuel reduction was performed and no more pathology was found. He was fed 2 days after operation and discharged 8 days after. He was free of any symptom for the last 6 months. Conclusion: Intussusception should be remembered in neonatal admitted with bilious vomiting and rectal bleeding besides necrotizing enterocolitis. Keywords: Intussusception, acute abdomen, newborn [P-070] To analyse effect of tracheal occlusion in the development of lung in nitrofen induced congential diaphragmatic hernia model Emrah Aydın Bahcelievler State Hospital Aim: Congenital daphragmatic hernia is a congenital abnormality with high morbidity and mortality. Lung hypoplasia and pulmonary hypertension is the leading cause of death. In our study we aim to analyse the effect of tracheal occlusion in the development of lung in nitrofen induced congenital diaphragmatic hernia. Method: Same age group Wistar albino rats were mated. Rats were included in the study whose vaginal smear showed sperm in. It was zero day of pregnancy in which sperm was shown in vaginal smear. At the 9th day of pregnancy 100mg Nitrofen was introduced to pregnant rats by gavage and at the 18thday of pregnancy laparatomy and hysterotomy was performed. Tracheal occlusion was performed one of two fetuses and the other one was accepted as control group. At 22nd day of pregnancy laparatomy and hysterotomy was performed and lungs of fetuses were excised. Development of lungs were analysed histopathologically. Results: Ten (%59) of 17 fetuses had congenital diaphragmatic hernia. Five of them were within control group and 5 were within surgical group. Defect of diaphragm was rigth sided in 5 of cases, left in 3 and bilateral in 2. Liver had tendency to toracic cavity in all. Development of lung was stopped in psödoglandüler stage in 2 of cases, in psödoglandüler-early canalicular stage in 2 and early canalicular in 1 of cases in control group while late canalicular-early saccular in 2, saccular in 1 and early canalicular in 2 of cases in surgical group. Conclusion: Nitrofen induced congenital diaphragmatic hernia is an efficient model but it cannot simulate Bochdalek’s hernia. Tracheal occlusion in nitrofen induced congenital diaphragmatic hernia model increases lung development. Keywords: Congenital diaphragmatic hernia, nitrofen, tracheal oclusion [P-071] Actual View to Diagnosis of Cystic Fibrosis Emre Özer1, Ayşe Ayzıt Atabek2, Fatma Deniz Uslu Aygün3, Haluk Çokuğraş2 1 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatrics, Istanbul, TURKEY 2 Istanbul University Cerrahpasa Medical Faculty, Department of Pediatrics Division of Pneumology, Istanbul, Turkey 3 Istanbul University Cerrahpasa Medical Faculty, Department of Pediatrics Division of Infectious Diseases, Istanbul, Turkey A six-weeks old girl who was treated for sepsis and meconium ileus was admitted to our hospital with symptoms of vomiting and cough. Physical examination - 46 - showed that she has sunken eyeballs, dry mouth, pale appearance. Her breathing sounded rough, crackles were present. Laboratory examination showed hyponatremic and hypochloremic metabolic alkalosis and the posterior anterior chest x-ray revealed the infiltration. The patent, who had high chloride levels in sweat twice, was diagnosed with cystic fibrosis and pneumonia. On the 14th day of treatment, the patient’s CF mutation analysis resulted as ∆F508 homozygosis, without any infiltration on the chest x-ray were observed due to the clinically significant improvement. Cystic fibrosis is a genetic disease which shortens life expectancy and impact the quality of life. This disease is characterized by the mulfunction of mucous glands primarily in lung and pancreas. The most significant issue for the patients is the recurrent lung infections and irreversible lung damage due to secretions which could not be removed, and malabsorption developed due to pancreatitis. Fundamental treatment of cystic fibrosis is the removal of dense lung secretions, treatment of infections, and the treatment of pancreatitis in additon to vitamin and nutritional support. However, in the recent years, the treatments, which remedy the failure of chloride channel, were developed and those studies are still ongoing. If the cystic fibrosis is diagnosed early and treated accordingly, both the life expectancy gets longer and the quality of life improves. In Turkey, it is aimed to prevent the delay of diagnosis and treatment by the newborn screening. This study aimes to emphasize the importance of the diagnosis and screening of cyctic fibosis and how the treatment should be implemented, by considering a newborn patient with the symptoms of meconium ileus and development of hypochloremic hyponatremic dehydration at 6-weeks. Keywords: Cystic fibrosis, immunoreactive trypsinogen, sweat test [P-072] Veno-occlusive disease as a complication of postoperative chemotherapy for Wilms tumor, successful and safe treatment with defibrotide Emre Tasdemir1, Gurcan Dikme2, Dilara Ece Toprak1, Emre Ozer1, Hande Kumkumoglu2, Begum Koc2, Ayse Ucar3, Sebuh Kuruoglu3, Tulin Tiraje Celkan2 1 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatrics 2 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatric Hematology Oncology 3 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatric Radiology Veno-occlusive disease (VOD) is an obliterative venulitis of the terminal hepatic venules. The clinical presentation of VOD includes jaundice, development of right upperquadrant pain, tender hepatomegaly, ascites and unexplained weight gain. VOD is a well-known early complication after human stem cell transplantation (HSCT), however a rare and severe complication of chemotherapeutic drugs. We report a 8 months old male infant with Wilms tumor stage 2 who developed VOD after second course of chemotherapy ( actinomicin and vincristine ). The patient presented with abdominal distension, weight gain, hepatomegaly, ascites, hyperbilirubinemia, severe thrombocytopenia, anemia, increase of liver transaminases and abnormalities of coagulation on the day of 10 days after second administration of actinomicin D. Defibrotide on a compassionate-use basis was started. The dosage was 15 mg/kg/day in 4 divided doses and VOD recovered after defibrotide treatment especially clinically with laboratory findings in 3th day. Defibrotide (DF) is a polydeoxyribonucleotide that has been found to have anti-thrombotic, anti-ischemic and thrombolytic properties without causing significant anticoagulation. There are limited reports on the use of defibrotide outside the human stem cell transplantation (HSCT) setting. Herein, we report a case with Wilms tumor complicated with VOD after chemotherapy which is rarely seen. Keywords: veno-occlusive disease, wilms tumor, defibrotide [P-073] Fibrinogen storage disease associated with hypobetalipoproteinemia and hepatocellular disease due to mutation in fibrinogen Aguadilla in a Turkish child Emrullah Arslan1, Tanyel Zubarioglu2, Gozde Yesil3, Ertugrul Kiykim2, Birol Ozturk4, Ayse Cigdem Aktuglu Zeybek2 1 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatrics 2 Istanbul University Cerrahpasa Medical Faculty, Department of Pediatrics Division of Nutrition and Metabolism 3 Bezmialem Vakif University, Faculty of Medicine, Department of Medical Genetics 4 Marmara University, Faculty of Medicine, Departement of Pediatrics Division of Gastroenterology Hepatic fibrinogen storage disease (HFSD) is a rare cause of chronic hepatocellular disease that is caused by mutations in the γ-chain of the fibrinogen (FGG) gene. It is characterized by hypofibrinogenemia and accumulation of abnormal fibrinogen within hepatocytes. In addition, hypofibrinogenemia is associated with hypobetalipoproteinemia in Aguadilla mutation. In this report, 3 years old male patient with elevated liver enzymes, hypolipoproteinemia and hypofibrinogenemia who was diagnosed as hepatic fibrinogen disease is reported. A 3 years old male patient was referred to our outpatient clinic with hepatomegaly. In his physical examination, liver was palpated 5 cm below the costal margin. Initial laboratory examinations revealed elevated transaminase levels, hypofibrinogenemia and dyslipidemia. Lipid electroforesis and apo B levels were compatible with hypobetalipoproteinemia. Immunomorphologic and electron microscopic studies showed selective and exclusive accumulation of fibrinogen within the endoplasmic reticulum in liver biopsy of the patient. Depending to laboratory and histological findings of the patient HFSD was considered in differential diagnosis of hepatocellular disease. Molecular analysis of FGG gene was performed and patient was found in heterozygote state for p.Arg401Trp mutation (fibrinogen Aguadilla). - 47 - HFSD is characterized by hypofibrinogenemia and accumulation of abnormal fibrinogen within hepatocytes. Despite its rarity, HFSD should be considered in differential diagnosis of chronic hepatocellular diseases especially in cases without an unknown etiology. Keywords: hepatic fibrinogen storage disease, hypobetalipoproteinemia, hypofibrinogenemia [P-074] A Rare Disease Presented with Abdominal Pain and Red Eye: Tubulointerstitial Nephritis and Uveitis Syndrome (TINU) Müferet Ergüven, Enise Avcı, Cengiz Candan, Pınar Turhan, Emine Olcay Yasa, Gizem Höyük, Betül Aluç Medeniyet University,Goztepe Education and Research Hospital, Department of Pediatrics, Istanbul Background&Aims: Uveal inflammation can rarely accompany tubulointerstitial nephritis that cause renal failure in children. This cooccurrence was first defined in 1975 and termed as tubulointerstitial nephritis and uveitis syndrome; TINU. Methods: A 16-year-old male patient has come with the complaints of abdominal pain and red eye. There was no history of chronic illness, drug use, arthritis, oral and genital ulcers, skin eruption. He was diagnosed with uveitis after membrane formation in the right eye was detected His laboratory findings suggested mild renal insufficiency. CBC was normal, ESH (64 mm/h ) and CRP ( 9,39 mg/dl) were increased. Serum urea (39 mg/dl) was normal and kreatinin (2,19 mg/dl) was increased, GFR was 63 ml/dk/1.73 m2. Urinalysis showed low urine density (1007), normoglycemic glycosuria (+++), and nonnephrotic proteinuria (+) In the 24-hour urinalysis, protein level was nephritic (13,44 mg/m2/h) and beta 2 mikroglobulin level was increased (25 mg/day). In further laboratory findings, ANA, Anti dsDNA, ANCA, RF were found negative. C3, C4, TSH, PTH, ACE levels were normal. The viral serology was unremarkable. PPD skin test and Patergy test had shown no reaction. A renal biopsy was performed and showed dense leukocytes in the interstitium. The patient was diagnosed with TINU syndrome. Both his uveitis and interstitial nephritis responded to high-dose corticosteroid treatment. Results: The definitive diagnosis of TINU syndrome is nonspecific and is suggested by the combination of uveitis and renal involvement, with renal biopsy consistent with acute interstitial nephritis. Patients with TINU are typically treated with prednisone at a dose of 1 mg/kg per day. Conclusions: The differential diagnosis for interstitial nephritis occurring in association with ocular findings is broad. The differential diagnosis includes sarcoidosis, Sjögren’s syndrome, systemic lupus erythematosus, Wegener’s granulomatosis, Behçet’s disease, and infectious diseases such as tuberculosis, brucellosis, toxoplasmosis, and histoplasmosis. Keywords: renal insufficiency, tubulointerstitial nephritis, uveitis [P-075] The Prolonged Fever in Early Childhood, Kawasaki Disease Erkan Erfidan1, Gökçen Kamış1, Mustafa Çakan2, Nuray Aktay Ayaz2, Helen Bornaun3, Hüseyin Aldemir1, Emel Karaoğlan1, Seda Balkaya1 1 Department of Pediatrics, Kanuni Sultan Suleyman Teaching Hospital, Istanbul, Turkey 2 Department of Pediatric Rheumatology Clinic, Kanuni Sultan Suleyman Teaching Hospital, Istanbul, Turkey 3 Department of Pediatric Cardiology Clinic, Kanuni Sultan Suleyman Teaching Hospital, Istanbul, Turkey Introduction: Kawasaki Disease is one of the most frequent causes of the acquired heart diseases affecting children in developed countries. More than 50% of the cases are between 6 months to 2 years old. Only 10% of the patients are younger than 6 months old. The incidence is very low through children younger than 3 months and older than 8 years old. Case Presentation: A thirty eight days old female is referred to our clinics with high grade fever and vomiting. She is hospitalized with the diagnosis of sepsis. Her physical examination findings are unremarkable. Laboratory investigations show WBC:16x103/µL, Plt: 351x103/µL, CRP:58mg/L, ESR:60mm/h. Urine analysis and chest X-ray are normal. The peripheral blood smear shows neutrophilia with toxic granulation, multiple platelet clumps. After blood, urine and CSF culturing, the antibiotic treatment is started as Ampicilina-Sulbactam and Cefotaxime. During the hospitalization, maculopapular rash appears in her cheeks,trunk and proximal extremities. erythema and fissure in her lips(Figure 1). Due to suspicion of the Kawasaki disease, Echocardiography is done, but shows coronary arteries with normal diameter. Incomplete Kawasaki disease cannot be ruled out, so IVIG (2 gr/ kg) is given and acetylsalicylic acid (4 mg/kg/day) is added. After a few days, periungal desquamation begins. In control echocardiography, right and left coronary arteries are measured respectively 1.24mm and 1.65mm (z-score:0.91). Second IVIG dose is given. Methylprednisolone (2 mg/kg/day) is added due to high fever. During the follow-up, the fever is reduced. So methylprednisolone is gradually stopped. Conclusion: Kawasaki disease should be remembered in toddlers with resistant fever to the antipyretics and antibiotics and significantly high acute phase reactants. İncomplete form is seen more often in early periods of the life. Early diagnosis and treatment may save the coronary arteries. Keywords: Kawasaki disease, early childhood, prolonged fever - 48 - [P-076] [P-077] Erkan Erfidan1, Gökçen Kamış1, Mustafa Çakan2, Nuray Aktay Akaz2, Hamide Sevinç Genç1, Sevgi Yavuz3, Aysel Kıyak3, Gönül Aydoğan4 1 Department of Pediatrics, Kanuni Sultan Suleyman Teaching Hospital, Istanbul, Turkey 2 Department of Pediatric Rheumatology Clinic, Kanuni Sultan Suleyman Teaching Hospital, Istanbul, Turkey 3 Department of Pediatric Nephrology Clinic, Kanuni Sultan Suleyman Teaching Hospital, Istanbul, Turkey 4 Department of Pediatric Hematology-Oncology Clinic, Kanuni Sultan Suleyman Teaching Hospital, Istanbul, Turkey Introduction: SLE is a chronic autoimmune disease. Presentation of a case may vary the mild disease with skin and joint involvement to serious disease with severe anemia, renal failure, convulsions. Hematologic involvement is almost seen in every patient. Mild to moderate anemia of chronic disease is the most commonly seen hematologic involvement. Autoimmune hemolytic anemia and thrombocytopenia is seen 2535%. Renal involvement is seen in half of the patients and mostly as class 4 lupus nephritis. In renal, cerebral and hematologic involvements, the treatment choice is steroids and monthly cyclophosphamide infusions. İf the patient is resistant to the classical treatment, second choice is rituximab that is a monoclonal antibody against the CD20 and cause apoptosis of B lymphocytes. Case Presentation: The patient who has been followed up for three years with SLE is admitted to the hospital with malaise. Three years ago, she diagnosed as Type 4 Lupus Nephritis and treated with six dose cyclophosphamide, then low dose steroids, hydroxychloroquine treatment was planned for maintenance. But she hasn’t used her drugs for last six months. Laboratory examination shows WBC:5300/mm3 Hb: 5.7gr/dL Hct:15.3% Plt:253000/mm3 LDH:850 U/L BUN: 22mg/dL Kreatinin: 0.7mg/dL Direct and indirect Coombs:positive. Bone marrow aspiration is normal. IVIG and hidh dose methylprednisolon treatment is started. During the treatment, platelet counts decreased and kidney function tests deteriorated. Cyclophosphamide infusion is given to the patient. Two weeks after the treatment, Hb:4.8 gr/dL PLT: 59000/mm3 BUN:36mg/ dL Kreatinin: 1.8mg/dL. The patient is resistant to treatment,so Rituximabe (375 mg/m2) is given as weekly for four weeks. After four dose, WBC:5000/mm3 Hb:11mg/dL Hct:37% Plt:189000/mm3 BUN:23mg/dL Kreatinin:0.9mg/dL Direct and indiret Coombs: negative. For maintenance of the treatment, low dose steroids, Mycophenolate mofetil, hydroxychloroquine are planned. Steroid and cyclophosphamide resistant SLE patients with severe hematological and renal involvement may respond to Rituximab treatment like in our case. Keywords: Systemic Lupus Erythematosis, Rituximab, Hematologic and Renal Involvement Erkan Erfidan1, Gökçen Kamış1, Mustafa Çakan2, Nuray Aktay Ayaz2, Hamide Sevinç Genç1, Zeynep Topkarcı3, Ayşe Kavak3 1 Department of Pediatrics, Kanuni Sultan Suleyman Teaching Hospital, Istanbul, Turkey 2 Department of Pediatric Rheumatology Clinic, Kanuni Sultan Suleyman Teaching Hospital, Istanbul, Turkey 3 Department of Dermatology, Bakirkoy Dr Sadi Konuk Teaching Hospital, Istanbul, Turkey Introduction: JDM is an extremely rare disease in childhood and primarily effects muscle and skin. Diagnostic criteria of the disease are typical skin lesions, symmetric proximal muscle weakness, high muscle enzymes, EMG and muscle biopsy results. For the diagnosis, three of the four criteria with typical skin lesions are enough. Skin lesions are pathognomonic in JDM and found 80-90% of the cases. Also heliotropic rash, Gorton papules and periungal changes, edema of the face and extremities are typical findings. Skin ulcers are found in 6-23% of the cases. But extensive and multiple skin necrosis like in our case, is reported in limited numbers. Steroids and methotrexate is first choice of the treatment in JDM. We discussed a case who developed extensive necrotic skin lesions under steroid and methotrexate and treated successfully with cyclosporin and monthly IVIG infusion. Rituximab Treatment of A Systemic Lupus Erythematosis Case with Severe Hematologic and Renal Involvement A case of Juvenile Dermatomyositis with Severe Skin Necrosis and Treatment with Cyclosporin and IVIG Case: A five years old female is admitted to the hospital with unable to climb stairs, unable to stand up, rash and edema on her eyelids and hands. Her physical examination revealed the proximal muscle weakness, periorbital heliotropic rash, edema in her face, Gorton papulles on metacarpophalangeal and proximal interphalangeal joints. İn capillaroscopy examination, capillary dilatation, decrease of the capillaries and micro bleedings are seen. Gastrointestinal system and lungs don’t involved. There is a ulceration about 2x2 centimetres on left scapula. CMAS score is 17. EMG examination shows myopathic changes. CK levels are 457 U/L (normal levels:30-155U/L), AST is 65 U/L. MR imaging of lower extremities show extensive myositis of all muscle groups. She is diagnosed as JDM and started to the treatment with methotrexate (15 mg/m2/week) and high dose methylprednisolon (30mg/m2/day). Cyclosporin and IVIG treatment may be an option for JDM patient with extensive necrotic skin lesions Keywords: Juvenile Dermatomyositis, Skin necrosis, Cyclosporin and IVIG - 49 - [P-078] Clinical outcomes of myocardial non compaction, is genetic testing necessary for all patients? Reyhan Dedeoğlu1, Funda Öztunç1, Erkan Erfidan3, Helen Bornaun2, Sezen Atik1, Aida Koka1, Levent Saltık1 1 Department of Pediatric Cardiology, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey 2 Department of Pediatric Cardiology, Kanuni Sultan Suleyman Teaching Hospital, Istanbul, Turkey 3 Department of Pediatrics, Kanuni Sultan Suleyman Teaching Hospital, Istanbul, Turkey Objective: Myocardial non-compaction (MNC) is a rare type of cardiomyopathy, resulting from the failure of myocardial development during embryogenesis. Methods: We retrospectively investigated records of 13 patients diagnosed as non-compaction cardiomyopathy. We used four echocardiographic characteristics of Jenni criteria. We also had detailed biochemical and metabolic analyses of each patient in addition to genetic testing. Results: 13 patients who were diagnosed with MNC were admitted to our clinic within the 2-years period. 8 patient had systolic dysfunction and suffered from heart failure, 3 of these also had pulmonary hypertension, 5 patient had normal cardiac function (CF). We identified two new genetic mutations for MNC but these patients were died during the neonatal period. One patient was diagnosed with Hallermann-Streiff Syndrome which was the first reported case associated with MNC. Genetic testing revealed MNC mutation in one of the patients which had MNC (heterozygot c.481G>T p.C161G mutation in ANKRD1 gene) but normal CF. All patients were on follow-up. Conclusions: This study demonstrates that mortality rate of MNC in newborns can be higher. Genetic testing should be suggested for each MNC patient even having normal CF for counselling future prognosis. Keywords: myocardial non compaction, genetic testing [P-079] Steroids secondary complication occuring in the treatment of juvenile idiopathic arthritis: ZONA ZOSTER Müferet Ergüven, Olcay Yasa, Ersin Böcü, Elif Yıldız İstanbul Medeniyet Üniversitesi Göztepe EAH Introduction: Herpes zoster infection occurs in the following trigeminal ganglia and it arises out of reactivated VSV which remains as latent in sensory neurons in the dorsal root. Generalized or disseminated herpes zoster can be seen in the immunocompromised patients or in cases of such secondary immunosuppresion of steroid use. Purpose: The case has been reported in order to emphasise the possible emergence of zona zoster infection caused by steroid used in the treatment of JIA patients. Case: 9-year-old female patient with the diagnosis of JIA applied to the pediatric rhuematology policlinic by the reason of itchy and painful vesicular lesions which began 3 days ago and spread from right lumbar region to dorsal. The patient having vesicular lesions was hospitalized with a diagnosis of zona zoster. Because of the JIA symptoms, the patient had received 1 mg/kg/day metilprednizolon and salazopyrin therapy during 3 weeks. In the physical examination, there were no active arthritis symptoms in the patient having normal height and weight percentile. There were vesicular zona lesions along the right thoracic dermatome. In the medical workup, WBC:14500 /mm3, CRP: 6.51 mg/dl, Sedimentation: 47 mm/h, biochemical values was fixed normal. 1500 mg/m2/day acyclovir IV, acyclovir cream, B vitamin PO, antihistaminic PO and IV hydration treatment was administered. Methylpredinisolone dose was decreased gradually. On the sixth day of treatment, pain and itching were at an end, and lesions started to regress.Additional pathology did not arise in the patient complated the treatment in ten days. Prophylactic oral acyclovir was prescribed to the patient, whose complains finished and lesions started to obviously regress, and the patient was treated. Conclusion: Steroid secondary zona zoster, which occurs as a complication on the sidelines of JIA treatment, can emerge. These patients follow-ups are important also for this aspect. Keywords: zona, steroid [P-080] Primary Spontaneous Pneumothorax Case at an Early Age Ayse Ayzit Atabek1, Esra Karabiyik3, Shiyar Hasan3, Fatma Deniz Aygun2, Haluk Cokugras1 1 Pediatric Pulmonology, Cerrahpasa Faculty of Pediatric Medicine, İstanbul University,İstanbul 2 Pediatric Infectious Disease, Cerrahpasa Faculty of Pediatric Medicine, İstanbul University,İstanbul 3 Cerrahpasa Faculty of Pediatric Medicine, İstanbul University, İstanbul Pneumothorax is defined as the existence of air in the pleural space between the lungs and chest wall. A 15-yearold female patient was admitted to the emergency unit due to the sudden onset of chest pain. In her physical examination, she looked pale and had tachypnea. When she was auscultated, it was found that breathing sounds was decreased in the left lung and cardiac sounds were deeply. After thoracic tube was inserted to the patient whose chest radiography revealed a pneumothorax line in the left hemithorax, her tachypnea regressed and oxygen saturation was elevated. In the tomography of the thorax subpleural cysts were detected in the apical segment of the upper lobe in the right lung and in the apicoposterior segment of the upper lobe in the left lung. Pleurodesis/ VATS was not thought to be performed because the patient did not have a history of previous pneumothorax and pneumothorax did not persist. Then, the patient, whose general state of health was good, was discharged from the hospital by giving some recommendations. Primary spontaneous pneumothorax(PSP) is the type of pneumothorax that occurs in patients without any - 50 - underlying pulmonary disease. It mostly develops due to the rupture of apical subpleural blebs. Patients are generally young, tall and slim males. The most important risk factor is smoking. The recurrence rate of this disease is high. The goal of treatment in the first episode is to decrease symptoms independently of the size of pneumothorax. Aspiration is needed only in symptomatic patients. On the other hand, talc pleurodosis or VATS (video assisted thoracic surgery) are the techniques that should be preferred in recurrent PSP.In this study, it was aimed to present a PSP patient, who applied with the complaint of chest pain, in order to re-evaluate the etiology and treatment of the disease. Keywords: Spontaneous Pneumothorax, VATS, Pleurodosis [P-081] Right Aortic Arch with Right Ductus Arteriosus Esra Özpınar1, Helen Bornaun2, Kazım Öztarhan2, Merih Çetinkaya3, Gökhan Büyükkale3, Seda Yılmaz Semerci3 1 Kanuni Sultan Suleyman Training and Research Hospital,Department of Pediatrics, İstanbul, Turkey 2 Kanuni Sultan Suleyman Training and Research Hospital,Department of Pediatric Cardiology, İstanbul, Turkey 3 Kanuni Sultan Suleyman Training and Research Hospital, Department of Neonatology, İstanbul, Turkey The term right aortic arch (RAA) with right ductus arteriosus (RDA) refers to a congenital abnormal position of the aortic arch, that is, to the right of the trachea, with or without an abnormal branching pattern, and is one of the least frequently prenatally diagnosed cardiac abnormalities. Right aortic arch identified in prenatal period is associated frequently with other cardiac/non-cardiac malformations, notably tracheal or esophageal compression and microdeletions 22q11. Intrauterine and postnatal survey of the fetus depends on these anomalies and their effects. The right aortic arch and double aortic arch have been known about for a long time although a right ductus arteriosus has however only occasionally been described. We describe here a case of RAA with a RDA in which with no other cardiac or morphological abnormalities. Keywords: right aortic arch, right ductus arteriosus [P-082] Trends and prevalence of overweight and obesity in school children from 2005 to 2014 in Turkey Mustafa Akcam1, Tugba Koca1, Ozgur Pirgon2, Selim Dereci1, Esra Sesli3 1 Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Suleyman Demirel University Medical School, Isparta, Turkey 2 Division of Pediatric Endocrinology, Department of Pediatrics, Suleyman Demirel University Medical School, Isparta, Turkey 3 Department of Pediatrics, Suleyman Demirel University Medical School, Isparta, Turkey Aim: The aim of this study was to determine the prevalence of overweight and obesity in school children and to compare the results with previous studies conducted by our clinic in the same region, in 2005 and 2009. Methods: The study was conducted at 7 primary schools and 3 high schools in Isparta, southern Turkey, which had been randomly selected for a study on obesity, 5 and 9 years previously. The height and weight of the students were measured in a standard way. Those in the 85-94 percent of BMI were classified as overweight and those >=95 percent were classified as obese. Results: The study comprised a total of 7116 students; 3445 (48.4%) females and with a mean age of 11.7±2.7 years (range, 5.8-18.9 years). The total of the overweight+obesity prevalence is determined as 23.5% for 2014 year. They were 23.8% and 23.5% for the years 2005 and 2009, respectively. No statistically significant difference was determined in the prevalence according to the years. The total overweight frequency was determined as 13.6% for 2014 year. They were 12.2% and 11.1% for the years 2005 and 2009, respectively. The total obesity rate was determined as 9.9% in 2014. They were 11.6% and 12.5% for the years 2005 and 2009, respectively. When the current study results were compared with those of previous studies, the overweight prevalence was seen to have increased and the obesity rates had decreased (p<0.05). Conclusion: Although the prevalence of overweight+obesity is still at a high level, it’s level shows not in increasing trend in recent years, it pauses, and even shows signs of a decrease in obesity, like in some developed country. It seems that through the efforts of recent years, there may be a promising trend towards reduction rather than increase. Keywords: obesity, children, overweight [P-083] Herpetic Whitlow in pediatric patient; a rare localization of primary herpes infection Esra Yücel1, Adem Karbuz2 1 SB Okmeydanı EAH Department of Pediatric Allergy and Immunology 2 SB Okmeydanı EAH Department of Pediatric Infectious Diseases Background&Aims: Primary herpetic infections of children are commonly localized in oral mucosa as herpetic gingivostomatitis. Primary herpetic whitlow is a rare condition in pediatric patients. Pediatricians should distinguish this from other infectious diseases (e.g bacterial whitlow) because of the different treatments are required. Here we present a 9 month-old boy with a three days history of painful swelling and multiple pustules in right index finger. Case: A 9 month-old boy is referred to our clinic because of fever, erythema, swelling and multiple pustules in his right index finger. He had been prescribed amoxicillinclavulonic, the lesion was diagnosed as bacterial whitlow. Despite this antibiotherapy his complaints persisted. He - 51 - was born 3360 as the first child of non consanginous parents. His neurologic, physical and social development is normal. When he admitted to hospital his fever was 38.2 C, his vital signs were in normal limits. In laboratory examination only mild leukocytosis was detected. His family reported that his baby sitter has also herpes labialis and the patient has finger sucking behavior for two weeks. According to this clinical condition herpetic whitlow was suggested, his antibiotherapy stopped and oral acyclovir were prescribed for one week. The patient lesions resolved within ten days. Conclusions: Primary herpetic whitlow is a rare condition generally caused by HSV 1. This is usually seen in children co-occur with herpetic gindivostomatitis or close contact to infected secretions of daily care givers. The longer course and failure to the antibiotherapy is definitive for herpetic whitlow. Pediatricians should be aware of herpetic whitlow as an occupational risk for themselves and also delayed and failed treatment puts on the patients at risk of superinfection and other complications. Keywords: Primary, HSV 1, Whitlow [P-084] Leukocyte Adhesion Deficiency-1(LAD-1) in a pediatric patient presenting with perirectal abscess Esra Yücel1, Saniye Berna Hamilçıkan2, Zeynep Gör2, Yeşim Acar3, Ömer Faruk Beşer3 1 SB Okmeydanı EAH Department of Pediatric Allergy and Immunology 2 SB Okmeydanı EAH Department of Pediatrics 3 SB Okmeydanı EAH Department of Pediatric Gastroenterology Leukocyte Adhesion Deficiency-1 (LAD-1) is a rare autosomal recessive primary immune deficiency characterised by the absence expression of the adhesion molecules on leukocytes. LAD-1 is associated with leukocytosis, recurrent bacterial infections, delayed separation of umbilical cord and impaired healing of wounds. Omphalitis, sepsis, gingivitis, periodontitis, skin ulcers, perirectal abscess are the other common clinical features of this disease. We present 3 year-old girl admitted to our clinic because of fever, recurrent oral ulcers and perirectal ulcer. Case: She was the third child of consanguineous parents. Her umbilical cord was separated at 17 days of life. She was admitted to hospital for several times because of pneumonia and also had recurrent oral ulcers. She referred to our clinic for intractable fever, perirectal ulcer and high leukocyte counts on CBC. In physical examination periodontitis, oral ulcer was detected. Rectal examination revealed perirectal ulcer with purulent secretion. Laboratory data showed leukocytosis 74500 /mm3 with neutrophilia (90%). Serum immunoglobulin (Ig) G, Ig M, Ig E, Ig A levels and Nitroblue tetrazolium (NBT) test were normal. She had normal T,B and NK cell numbers in peripheral blood flow cytometric analysis but expression of adhesion molecules on leukocytes were absent (CD 18; 0%, CD11a; 3%, CD 11b; 0%, CD11c; 0%). 12*6 mm perirectal abscess was shown on pelvic MRI. She was received broad-spectrum antibiotics (sulperazone with metronidazole) for two weeks and discharged after recovery. Prophylactic oral amoxicillin was started. Conclusion: Different clinical manifestations have been reported in LAD-1 patients. Severity of symptoms is releated to the degree of surface expression of CD18 and CD11. Curative treatment is only possible with until hematopoietic stem cell transplantation (HSCT). Patients should receive antibiotics prophylaxis for infections until HSCT. Keywords: Leukocyte adhesion, perirectal ulcer, LAD-1 [P-085] Suspect hair tourniquet syndrome in crying infants without an obvious reason Engin Çarkçı1, Mehmet Esat Uygur2, Ethem Ayhan Ünkar3, Zeynep Alp Ünkar4 1 Department of Orthopaedics and Traumatology, Sefaköy Hospital, Medipol University, Istanbul, Turkey 2 Department of Orthopaedics and Traumatology, Vera Hospital, Istanbul, Turkey 3 Department of Orthopaedics and Traumatology, Kartal Yavuz Selim State Hospital, Istanbul,Turkey 4 Department of Pediatrics, Division of Neonatology, Medical Faculty, Marmara University, Istanbul, Turkey Background&Aims: Hair-tourniquet syndrome (HTS) is a rare and painful condition occurring when hair or thread pieces are tightly wrapped around toes, fingers or other appendages, occluding blood flow and causing strangulation or autoamputation. Our aim is to present a HTS and discuss possible reasons of it. Case: Four-month-old female infant was brought to our emergency clinic because of inconsolable crying. She was afebrile and without evident signs of systemic infection. Detailed physical examination after removing all clothing items revealed a hair thread circumferentially wrapped around the left 3rd toe at the level of proximal interphalangeal joint (Figures 1a and 1b). The distal part of the toe was strangulated and congested. The hair was removed under local anesthesia. Examination of the patient’s clothes revealed long strands of hair in her socks. Following removal of the strangulating hair, pain was completely relieved within 12 hours; whereas, recovery of the skin was prolonged up to 3 days. Discussion: HTS which was first defined in 17th century, is a rare entity not commonly known by both pediatricians and orthopedic surgeons. Even though child abuse should be kept in mind, the most common reason is accidental knotting of mothers hair around the infant’s toes. It is most commonly seen between the 2nd postnatal week and 6 months of age. The proposed reason is telogen effluvium, a form of nonscarring alopecia characterized by diffuse hair shedding. It can be caused by medications or by a metabolic or hormonal changes, especially in the postpartum period. HTS is diagnosed by physical examination. Digital infections can be ruled out by normal acute phase reactants. It should be suspected in cases of crying babies without an obvious reason, before diagnosing - 52 - infantile colic. Treatment is simple but if the oppurtunity is missed, it can lead to finger/toe deformities or amputation. Keywords: hair-tourniquet, toe, infant [P-086] The use of vascularized fibular graft in reconstruction of a long bone defect caused by osteomyelitis in a pediatric case Tolga Tüzüner1, Ahmet Şenel1, Ziya Demirci1, Ethem Ayhan Ünkar2, Zeynep Alp Ünkar3 1 Department of Orthopaedics and Traumatology, Istanbul Training and Research Hospital, Istanbul, Turkey 2 Department of Orthopaedics and Traumatology, Kartal Yavuz Selim State Hospital, Istanbul,Turkey 3 Department of Pediatrics, Medical Faculty, Marmara University, Istanbul, Turkey Introduction: Acute/subacute osteomyelitis causes serious morbidity in children because of delays in diagnosis and treatment. It may occur via hematogenous spread, invasion from peripheral tissues, or contamination from traumatic or surgical borders. Antibiotic regimens according to antibiograms are an important part of treatment along with surgery when necessary. Case: 8-year-old boy was operated for displaced subtrochanteric femur fracture. Osteosynthesis with internal fixation was applied. On the 15th postoperative day, discharge from a fistula on the operation region was observed. Empirical cefazolin treatment was started and wound culture was taken revealing Gram positive rods. Radiological findings were consistent for osteomyelitis. Plaque removal and debridement was performed. Postoperatively he stayed in intensive care unit (ICU) for one day. In the following days, a second debridement was performed due to rising acute phase reactants (APR). Debridement material culture was positive for P.aeruginosa; thus, antibiotherapy was changed to ceftazidime and amikacin. With decreasing APR, amikacin was stopped on the 14th day and ceftazidime was continued for another 4 weeks. At the end of treatment period, patient was asymptomatic and APR were negative. Fixation with a vascularized autologous fibular graft was performed to the defective pseudoarthrosis area caused by the early implant removal because of infection on the initial fracture line. In follow-up visits, healing in the graft area was observed and laboratory findings were normal. After physiotherapy, patient returned to his daily life. Conclusion: Surgery for femur fractures in pediatric age group is a crucial option with osteomyelitis as a rare but important complication. Patient’s stay in ICU might have caused contamination with resistant bacterial species, complicating healing and necessitating prolonged antibiotiotherapy. Biological reconstructive interventions have significantly improved the clinical outcome in pediatric patients. In literature, these kinds of interventions were more commonly performed after osteosarcoma resections but rarely in cases of defective pseudoarthrosis. Keywords: osteomyelitis, vascularized fibular graft, pseudoarthrosis [P-087] Short term results of computer assisted external fixator in treatment of neglected clubfoot deformity Tolga Tüzüner1, Ahmet Şenel1, Ziya Demirci1, Cenk Ermutlu1, Ethem Ayhan Ünkar2, Atakan Telatar1, Mustafa Güngör1 1 Istanbul Training and Research Hospital 2 Kartal Yavuz Selim State Hospital Background&Aims: Treatment of neglected or inadequately treated clubfoot deformity is quite problematic. Several corrective surgical procedures have been defined to gain painless plantigrad gait. Soft tissue complications and risk of neurovascular insult have made distraction osteogenesis and gradual correction favorable over acute procedures. We present the early results of 4 patients with complex foot deformities treated with computer assisted smart circular fixator in the last two years. Methods: Lengthening of the Achilles tendon, plantar fasciotomy and release of the posterior capsule was performed in all cases. One patient had osteotomy at the midtarsal level and another one had calcaneal osteotomy. Functional outcome is assessed according to criteria described by Ferreiara et al. Results: Patients’ ages ranged between 12 and 16. Two of the patients had congenital clubfoot whereas the other 2 cases had neurogenic etiology. One of the patients with clubfoot deformity was a totally neglected case while the other patient was initially treated in another country long time ago. One patient had neurological sequel from head trauma while the other case had meningomyelocele. Mean follow-up was 8 months. In one case, gradual correction had to be stopped and fixator was removed because of metatarsal fracture and infection. The patient has recovered from infection and is currently under medication for severe osteopenia.Two patients had good functional outcome while one patient had bad outcome. Conclusions: Infinite hinge points principle of computer assisted hexagonal external fixator devices make them advantageous over conventional Ilizarov designs for correcting neglected and complex foot deformities. However, treatment of these deformities is still problematic and prone to complications. Patient compliance, surgeon’s experience and steep learning curve should also be taken into consideration when attempting these corrections. Keywords: neglected, clubfoot, fixator - 53 - [P-088] [P-089] Reconstruction of radius and ulna following infectious nonunion with segmental bone loss in a pediatric case using non vascularised fibular graft Hemolytic Uremic Syndrome caused by coinfection of Salmonella Typhi and Escherichia Coli Tolga Tüzüner1, Ahmet Şenel1, Cenk Ermutlu1, Ziya Demirci1, Ethem Ayhan Ünkar2, Alican Barış1, Enes Kanay1 1 Istanbul Training and Research Hospital 2 Kartal Yavuz Selim State Hospital Background&Aims: Infection after internal fixation of fractures is rare in childhood. This complication is even less frequent in fractures of the both bones of the forearm where there is rich soft tissue vascularization. We present a case with severe infection and resultant bone destruction following plate fixation of Radius and ulna. Methods: Fourteen years old child with infective nonunion and bone loss of radius and ulna was referred to our clinic. He had external fixators applied to both bones. Patient history revealed open reduction and plate fixation of both bones 5 months prior to presentation. Plates had to be removed because of early onset suppurative infection. Laboratory was consistent with infection. He was diagnosed with osteomyelitis with massive bone loss and two stage reconstruction was planned. External fixators were left in place, extensive debridement was carried out and nonviable bone segments were resected en bloc. Antibiotic impregnated spacer was placed along the bone defect. Second stage of the treatment was begun after 6 weeks of parenteral antibiotics treatment following return of inflammatory markers to normal levels. Bone defects were reconstructed using nonvascularised fibular graft fixed with compression plates. Results: Early postoperative radiographs revealed that original lengths of both bones were restored. Graft incorporation was evident on plain x-rays at month 1 and 3. He had full wrist and elbow ROM at the final follow-up. Conclusions: Osteomyelitis of the both bones of the forearm in the pediatric population is extremely rare. Simultaneous inoculation of both bones despite exposure through two different incisions make hematogenous spread more probable. Two staged treatment of implant related infections is a widely accepted approach. Reconstruction of massive bone loss following infection is controversial. In pediatric patients, use of nonvascularised fibular grafts is more suitable due to technical simplicity and high rate of success. Keywords: childhood, nonvascularized, reconstruciton Ezgi Yalçın, Nilüfer Göknar, Ayşegül Doğan Demir, Faruk Öktem Bezmi Alem Vakıf Üniversitesi Tıp Fakültesi Background&Aims: Hemoltyic-uremic syndrome is one of the most common cause of acute kidney injury in young children.It is characterized by the triad of microangiopathic hemolytic anemia,thrombocytopenia,and renal failure. The most common form of HUS is diarrhea related type and the etiologic factors are shigella toxin-producing Escherichia Coli (STEC), Shigella dysenteriae and rarely Salmonella Typhi.In this case we presented a patient with HUS co-infected by Salmonella and STEC. Methods: A previously healthy 7-year-old girl was admitted to our emergency room with abdominal pain, vomiting and darkening of the urine color.Laboratory tests demonstrated anemia, thrombocytopenia and elevated creatinine and BUN levels.A blood film revealed fragmented erythrocytes. She was diagnosed as Hemolytic Uremic Syndrome.After hospitalization she received hemodialysis with a continuous monitoring of hemoglobin levels and platelet counts. On second day of administration she had fever as 39C with axillary method. Salmonella Typhi was identified in the blood culture.Clinical improvement was observed after the start of ceftriaxone for salmonella.On follow up days, patient had positive stool culture for enterohemorrhagic E.coli.She was treated with intravenous ceftriaxone.On fourth week of hospitalization she was out of dialysis and the creatinine,hemoglobin and platelet levels slowly turned back to normal limits.She was discharged and received follow-up care as an outpatient. Results: Salmonella is a microorganism involved in the etiology of HUS but not very often encountered in clinical practice.In our case firstly we isolated S.thyphi from blood culture and then STEC from stool samples.Few cases in the literature have been reported co-infected by Salmonella and E.coli.According to Ardissino G et al. STEC is often(35.6%–39.9%) co-infected with either Salmonella and should be consider as STEC-associated HUS with a co-infection Conclusions: As a result,we consider that the Salmonella coinfection in patients with Hemolytic Uremic Syndrome also be kept in mind.This case was reported because of a rare co-occurence and high morbidity associated with the condition. Keywords: Hemolytic Uremic Syndrome, co-infected by Salmonella and E.coli - 54 - [P-090] [P-091] Fatih Varol1, Ayşe Ağbaş2, Beyhan Tüysüz3, Rumeysa Yasemin Çiçek2, Firuze Erbek Alp4, Nur Canpolat2, Halit Çam1 1 İstanbul University Cerrahpaşa Medical Faculty Pediatric Emergency Department 2 İstanbul University Cerrahpaşa Medical Faculty Pediatric Nephrology Department 3 İstanbul University Cerrahpaşa Medical Faculty Pediatric Genetics Department 4 İstanbul University Cerrahpaşa Medical Faculty Pediatrics Department Osteopetrosis with renal tubular acidosis (RTA) is a rare disease due to carbonic anhydrase (CA) II deficiency in which findings of both proximal and distal renal tubular dysfunction and extrarenal findings. We present a case with hypokalemic paralysis who was diagnosed RTA and osteopetrosis. 7 years old boy attended to emergency department with muscle weakness. He had been diagnosed as RTA with the findings of both proximal and distal renal tubular dysfunction including a normal anion gap metabolic acidosis with alkaline urine (urine pH as 7.0), hypouricemia, hypocitraturia, hypercalciuria, and nephrocalcinosis at the age of 3 years. Serum potassium level was in normal range during 4 years of follow up. In the admission, he was lethargic, unable to hold his head and walk. Muscle strength was reduced and he had growth retardation and hepatosplenomegaly. Serum potassium level was 1.4 mmol/L. There was ST depression, flattening of T wave on ECG. His X-Ray examination showed increased bone density without corticomedullary differentiation, broadened metaphysis and transverse fractures of tibia. CT scanning demonstrated hydrocephalus, cerebral calcification, crowded posterior fossa, hydrosyringomyelia, and osteopetrosis. On the basis of these findings, CA II deficiency was suspected. He was treated with potassium citrate. His neuromuscular findings were gradually resolved after potassium replacement. Hypokalemia should be remembered for differential diagnosis of muscle weakness and CA II deficiency should be kept in mind when the patient has the combination of RTA and osteopetrosis. Keywords: Osteopetrosis, hypocalemıc paralysis, rta Fatih Varol1, Fatih Aygün2, Reyhan Dedeoğlu3, Feyza Aydın4, Sevinç Mertoğlu4, Halit Çam1 1 İstanbul University Cerrahpaşa Medical Faculty Pediatric Emergency Department 2 İstanbul University Cerrahpaşa Medical Faculty Pediatric İntensive Care Department 3 İstanbul University Cerrahpaşa Medical Faculty Pediatric Cardiology Department 4 İstanbul University Cerrahpaşa Medical Faculty Pediatrics Department Hair-thread tourniquet syndrome is a rarely encountered disease. It is seen primarily in extremities but other body parts such as penis, clitoris and vulva can also be involved. Hair-thread tourniquet syndrome occurs when these body parts are strangulated with a strand of hair or a piece of thread. Early diagnosis and treatment prevents loss of involved sites. In this study we report a patient who presented with irritability due to toe strangulation with a strand of hair. Four-month-old male patient presented to us with a twoday history of irritability along with redness and swelling on his left toe. The past medical history and family history were insignificant. In physical examination a circular constriction line was observed on proximal phalanx of the fifth toe of the left foot with a strand of hair hanging from the constriction line. No other particularity was noted in physical examination. In the operation, which was performed by the plastic and reconstructive surgery department, the strand of hair was removed and circular sutures were put on the constriction line. Due to the facts that the skin and subcutaneous tissues were cut by the strand of hair and the constriction line reached the bone, the patient was put under parenteral antibiotics to prevent a possible osteomyelitis. After a further detailed history was taken it was understood that there was no child abuse. The family was informed about childcare and hygiene. The toe healed without sequela. Hair-thread tourniquet syndrome as its name suggests, is the condition in which a body part is strangulated with a strand of hair or a piece of thread. Mostly in extremities the fingers are involved; but other body parts such as penis, clitoris and vulva can also be involved. In every case, child abuse should also be considered. Keywords: hair-theread tourniquet, strangulation Hypocalemıc Paralysis In A Case With Osteopetrosis With Renal Tubuler Acidosis Hair-thread tourniquet syndrome in a fourmonth-old male patient - 55 - [P-092] [P-093] Tuberculous Meningitis Presenting With Different Clinical Stages: Two Case Reports Fatma Nur Öz1, Mehmet Yusuf Sarı2, Hatice Gamze Poyrazoğlu3 1 Fırat University Medical Faculty, Ankara, Turkey, Division of Pediatric Infectious Diseases 2 Fırat University Medical Faculty, Ankara, Turkey, Division of Pediatric Intensive Care Unity 3 Fırat University Medical Faculty, Ankara, Turkey, Division of Pediatric Neurology It is estimated that one-third of the world’s population has been infected with Mycobacterium tuberculosis, and 70,000 individuals have tuberculous meningitis (TM). The clinical stage at the time of presentation is the single most important predictor of clinical outcome. Case 1: Fourteen years old male was referred to our hospital diagnosis of meningoencephalitis and hydrocephaly. His chilhood vaccination was absent. On examination his conscious was lethargic with a Glasgow coma score of 8/15 (E2V2M4). Cerebrospinal fluid (CSF) examination showed white cell count 100/mm³ (%80 lymphocyte), CSF protein 238 mg/dL, CSF glucose 23 mg/ dL. CSF to blood glucose ratio was reduced. No acid-fast bacilli (AFB) were seen in microscopy. CSF mycobacteria PCR was positive. A brain CT showed dilatation of all ventricles. Chest X-ray showed right middle zone consolidation. The patient was started on antituberculous drugs and intravenous steroids. His hydrocephaly was worsened progresively and he underwent VP shunt but his clinical condition deteriorated and he died on the sixth day after admission. Case 2: Sixteen yaers old female presented with headache, vomiting lasting for 1 week. She had no BCG vaccination. Her physical examination was normal except the signs of meningeal irritation. CSF examination showed 80/mm³ lymphocyte, CSF protein 60 mg/dL, CSF glucose 50 mg/dL, CSF/Blood glucose ratio was 0,4. The CSF microscopy were negative but AFB were seen. CSF mycobacteria PCR was positive. Chest X-ray showed right lower zone consolidation. Kranial MR showed mild hydrocephalus. The patient was started on antituberculous drugs and intravenous steroid. She made a remarkable clinical recovery and was discharged home 4 weeks after admission. Conclusion: We report two cases of TM, one of them presented late and rapidly developed hydrocephalus the other one has early presentation. This report is intended to make clinicians aware of clinical stages at presentation of TM. Keywords: tuberculous meningitis, children, hydrocephaly Successful treatment of a multidrug resistant Acinetobacter Baumannii wound infection in a patient with ewing sarcoma Fatma Özgüç Çömlek1, Nurhan Aruçi Kasap1, Özden Türel2, Fatma Betül Çakır3 1 Bezmialem Vakıf University Medical Faculty, Paediatrics, Turkey/Istanbul 2 Bezmialem Vakıf University Medical Faculty, Paediatric Infections,Turkey/Istanbul 3 Bezmialem Vakıf University Medical Faculty, Paediatric Hematology and Oncology,Turkey/Istanbul Summary: Nowadays, the increasing resistance to antibiotics that are used for treatment of Acinetobacter baumannii infections became a serious health problem in our country as well as all over the world. A. baumannii causes serious nasocomial infections and outbreaks. It quickly develops resistance therefore a combination of drugs is required for treatment. Here we describe a ewing sarkom patient with A. Baumannii wound infection and discuss treatment options in A. Baumannii. Case: A sixteen year old female patient diagnosed with Ewings Sarcome came to us. She had a wound infection in her primer tumor location. Laboratory findings showed CRP 25,1 mg/dL, WBC 22,57. Wound drainage was green and smelly. The treatment was continued by using meropenem and vancomycin. Wound, urine and blood materials were collected to make culture. After surgical consultation, it was not considered any invasive procedures only wound care was recommended with epitelizan creams. Five days after hospitalization was produced A. Baumannii which is multidrug-resistant, colistin and tigecycline sensitive in the wound culture and the treatment was replaced with colistin and ampicillin-sulbactam. Daily wound dressing was made. 7th and 15th days after treatment were performed wound cultures and the same bacteria was produced again. Control culture was negative on 27th day after treatment. Our patient has fully healed. The Patient was discharged and was asked to carry on with the treatment for the primer disease. Discussion: Acinetobacter baumannii is a nonfermantative, gram-negative, non-moving, catalase positive, oxidase negative microorganism. While these bacteria microscopically are seen as gram-negative bacilli(1) Recently, It is extensively encountered with Acinetobacter baumannii in nosocomial infections, such as meningitis, pneumonia, VAP, infection related to central venous catheter, urinary system infection, wound infection. Carbapenems, polymyxin E and B, sulbactam, piperacillin/ tazobactam, tigecycline and aminoglycosides, which are known effective against A. Baumannii, are being preferred from antibiotic agents. (2) Keywords: A.Baumannii, nosocomial infection - 56 - [P-094] Spontaneouse pneumomediastinum with subcutaneous emphysema in an adolescent boy Filiz Serdaroglu1, Tugba Koca2, Isa Döngel3, Fatma Saltık1 1 Department of Pediatrics, Süleyman Demirel University Faculty of Medicine, Isparta, Turkey 2 Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, Süleyman Demirel University Faculty of Medicine, Isparta, Turkey, 3 Department of Thoracic Surgery, Süleyman Demirel University Faculty of Medicine, Isparta, Turkey Spontaneous pneumomediastinum, which generally develops as a result of alveoli bursting under pressure, is rarely seen in children and may cause families and physicians to panic. Acute or chronic pulmonary disease is expected in the etiology. However, it may also be seen with exercises which increase pulmonary pressure, without any facilitating factor. The case is here presented of an adolescent with no known pulmonary disease, who presented with chest pain that had started during the normal activity of a game and the diagnosis of spontaneous pneumomediastinum was missed in the first physical examination. Keywords: Children, spontaneous pneumomediastinum, subcutaneous emphysema [P-095] Small bowel intussusception presenting as seizure and lethargy Fatouma Khalif Abdillahi1, Ufuk Erenberk2 1 Bezmialem Vakıf University, Department of Pediatrics 2 Bezmialem Vakıf University, Department of Pediatric Emergency Case Report: A previously healthy 1year-old boy is brought to the emergency department with a suspected seizure and letargy. The patient’s temperature was 37°C rectally, pulse 125, respiration 35, and blood pressure 100/80 mmHg. There were no signs of meningitis or intracranial hypertension, no abnormalities in sensibility and strength, and reflexes were normal. Cardiopulmonary examination revealed no heart murmurs, and breath sounds were normal. The abdomen was not painful to palpation and there were no masses. The laboratory studies were normal, including a white blood cell (WBC) count of 11,000,negative acute phase reactants,normal serum electrolytes and blood lactate as well as ammonia. Cranial MRI was planned. Air-fluid levels patern was perceived in abdominal plain film. The transabdominal ultrasound demonstrated a midabdominal targetshaped mass consistent with intussusception. Barium enema confirmed the diagnosis of intussusception, which was reducible in the radiology suite. The patient spent discharged to home after three days. Discussion: İntussusception is a common disease that can present with nonspecific symptoms, and may mimic a spectrum of serious illnesses. The findings of profound lethargy and seizure may lead the clinician towards a diagnosis of intracranial pathology. Fortunately, ED ultrasonography provides quick and accurate assessment of intraabdominal pathology that may be confounded by another process, or masked by vague presenting symptoms. The etiology of this alteration in status mental is unknow. Some hypothesis are considered to explain these clinical signs and symptoms by the realese of cytokines produced during the disease.İn our case, the child’s neurological state returned to normal immediately after proper blood supply to the bowel had been restored.The condition should be suspected in all children presenting with the association of seizure and lethargy if no cause is identified. Radiological or tomographic study of abdomen should be performed also in cases without intussusception typical symptoms. Keywords: Intussusception, lethargy, seizure [P-096] A Case: Hypothyroidy In Pediatric Secondary Pseudotumor Cerebri Syndrome Fayize Maden, Selvinaz Edizer, Aycan Unalp, Gurkan Gurbuz, Kazim Coban Dr. Behcet Uz Children’s Hospital Introduction: Idiopathic intracranial hypertension (IIH) also known as pseudotumor cerebri is characterized by signs and symptoms of increased intracranial pressure in the absence of a space-occupying lesion. The causes of IHH are numerous and include metabolic disorders (galactosemia,hypoparathyroidism, hypophosphatasia, prolonged corticosteroid therapy, possibly growth hormon treatment, malnourished child, hypervitaminosis A, severe vitamin A deficiency, obesity, menarche, oral contraceptives), infections,drugs (doxycycline, minocycline,tetracycline), hemotologic disorders (polycythemia, hemolytic and iron-defiency anemias), obstruction of intracranial drainage by venous thrombosis, head injury, and obstruction of the superior vena cava. Case Presentation: 15-year –old girl has had a headache for 2 years. Migraine was diagnosed and analgesic therapy was taken. The last 2 weeks raging and increased frequency of headache, she has and papilledema with an enlarged blind spot. Psoudotumor cerebri was diagnosed and she was hospitalized. Physical examination, her weight was over the 97 percentile and papilledema was revealed. The other system examination was normal. In order to diagnosis and treatment, lumbar puncture was performed, CSF pressure measured 30 cmH2O. Laborotary findings are; increased thyroid autoantibodies and TSH, normal T3, T4 were tested and thyroid ultrasound was interpreted in favor of thyroiditis. With these findings, she was referred endocrinology and she was started L-tyrosine treatment together acetazolamide. Conclusion: IHH is a diagnosis of exclusion. This is required to exclude all other causes of papiledema and raised intracranial pressure. We advise considering hypothyrodism as a potential cause of IHH. Also obesity accompanies with IHH. We see a strong association between IHH and recent weight gain. In thıs case, we specified that obesity related to hypotroidsm, may cause the pseudotumor cerebri. Keywords: Pseudotumor cerebri, Hypothyrodism - 57 - [P-097] It may not only acute pericarditis; myopericarditis in 3 adolescent boy Ferda Gürkan, Ali Yurtseven, Yasemin Özdemir Şahan, Raziye Burcu Güven Bilgin, Eylem Ulaş Saz Ege University School of Medicine, Department of Pediatrics, Division of Emergency Medicine Background&Aims: Acute pericarditis is inflammation of the pericardium defined as at least 2 of the following criteria: usually precordial or retrosternal chest pain (sharp and pleuritic, improved by sitting up and leaning forward), pericardial friction rub, suggestive ECG changes (generalized elevation of the ST segment or PR depressions), new or worsening pericardial effusion. Changes in myocardial necrosis markers (CK-MB and troponin) may occur for epicardial impairment and should suggest a diagnosis of myopericarditis. In up to 70 % of pediatric patients a specific etiology cannot be detected and myopericarditis is considered idiopathic. Methods: The medical records of 3 consecutive patients with acute pericarditis who presented to the emergency department were reviewed retrospectively. Results: Our patients were all male and had upper respiratory tract infection with subfebrile temperature at disease onset. According to their presentation time patients were 15, 17 and 16 years old, respectively. They all presented with retrosternal pleuritic chest pain and had suggestive ECG changes which were identified as following; patient 1 (elevation of the ST segment in DI, DII), patient 2 and 3 (elevation of the ST segment in V5,V6). The mean troponin,CRP, ESR level was 0.364ng/ ml (normal <0.014ng/ml), 3.6mg/dl and 30mm/h respectively. Echocardiogram showed that entire patients had minimal pericardial effusion. Patient 1 and 3 was treated by Ibuprofen, IVIG and ibuprofen used for patient 2. Myopericarditis was idiopathic or viral in 100 % of cases. Conclusions: Acute myopericarditis is most often either idiopathic or viral. The diagnosis is based on clinical criteria and laboratory testing. The evaluation and treatment of patients with acute pericarditis can be performed in the ambulatory setting unless the patient has myopericarditis. In most patients, acute pericarditis has a brief and benign course and an excellent response to NSAIDs, alone or in combination with other agents. Keywords: adolescent, myopericarditis [P-098] Dyskinesia and Kranial MRI changes caused by vitamin B12 in a 1 year old child Hüseyin Kılıç1, Feyza Aydın2, Sema Saltık1 1 Cerrahpaşa Tıp Fakültesi Çocuk Nöroloji Anabilim Dalı, İstanbul 2 Cerrahpaşa Tıp Fakültesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, İstanbul Introduction: B12 vitamin deficiency of first the years of the life generally arises from malnutrition and depleted maternal stores. Deficiency of this vitamin may cause to hematologic, neurologic and psychiatric disorders.The aim of this article is an evaluation of the development of encephalopathy-like clinical symptoms in a 1 year old child with B12 insufficiency and the cure of the clinical and radiological signs of the dyskinesia-like complications occurring after B12 vitamin replacement. Case: 12 months old healthy girl was born as a second child of healthy parents, with no consanguineous marriage and familial metabolic disorders history was admitted to the hospital due to somnolence, inability to feeding. In cranial magnetic resonance imaging (MRI), acute diffusion restriction and hyperintensity in the T2 sequences were detected on the inferior aspect of the bilateral lentiform nucleus. Very low level of B12 vitamin ( 30pg/ml) and anaemia was detected and B12 was replaced as a possible cause of these clinical signs. Symptoms were resolved after replacement and the patient was discharged.This patient was admitted to the hospital after one month with complaints of dyskinetic movements in her face and arms. There was no any other pathology besides dyskinesia and these symptoms were resolved after intravenous midazolam treatment. After B12 replacement serum B12 level was 1496 pg/ml and any pathologic finding was not found in metabolic and toxic analysis and in EMG of this patient. Maternal serum B12 level was low, as well (135 pg/ml). The patient was discharged with clonazepam treatment. In her control observation, dyskinetic symptoms were declined totally and clonazepam treatment was ceased. MRI signs were resolved in a control assessment after three months, as well. Conclusion: In a clinical scenario with a basal ganglionic involvement in the first years of the life, B12 depletion also should be taken into account. Keywords: vitamib b12, diskinezia [P-099] Vitamin B12 deficiency in childhood Sinan Yavuz, Ozgul Yigit, Meltem Erol, Zubeyde Kilinc Yaprak, Dilara Fucucuoglu, Feyza Coskun Bagcilar Training and Research Hospital, Pediatric Department, Istanbul, Turkey Background&Aims: Vitamin B12 deficiency is usually evoked in presence of compatible hematologic or clinical (usually neurologic) signs. Clinical findings are usually characterized by anemia, leucopenia, thrombocytopenia, anorexia, irritability, failure to thrive, developmental delay or regression, cognitive dysfunction and irreversible neurological damage. However, many cases of deficiency are asymptomatic. In this study we aimed to screen clinical and hemotological findings of the children determined vitamin B12 deficiency (incidentally or on clinical suspect). Methods: Clinical and laboratory features screened from medical automation system retrospectively. Clinically features (age, gender, complaints) and laboratory findings (hemoglobin, hematocrit, mean corpuscular volume, ferritin, serum iron levels, total iron binding capacity) are recorded and evaluated Results: In our study 127 patient included. Of patients %53 were female(n=67) %47 were male (n=60). The - 58 - main complaints of patients were paleness in %48, flu like symptoms in %31, failure to thrive in %18. Vitamin B12 deficiency is more frequently seen in groups aged 0-2 and aged 12-17. We determined mean vitamin B12 values 168.1±34.1 pg/ml, mean hemoglobulin values 12±1.9 g/dl, mean MCV values 78.4±8.1 fl. The hemotological features were anemia in %38, bicytopenia in %4, thrombocytopenia in %9 pancytopenia in %3, macrocytosis in %2 of patients. There is no significant difference in mean hemoglobin and RDW levels between the patients with low iron levels and normal ones. Conclusions: Vitamin B12 deficiency are primarily investigated in patients with anemia and neurological symptoms. Based on the knowledgement that vitamin B12 deficiency can be asymptomatic we suggest to investigate vitamin B12 levels especially in the ages of increased growth rate. Keywords: Childhood, Vitamin B12 deficiency [P-100] Hereditary Angioedema Case Report Feyza Coşkun1, Hikmet Tekin Nacaroğlu1, Meltem Erol1, Özgül Yiğit1, Raif Coşkun2 1 Ministry of Health Bağcılar Research and Training Hospital, Pediatric Clinic 2 Istanbul University, Faculty of Medicine, İnternal Disease, Department of Allergy and Immunology Background&Aims: Hereditary angioedema is an autosomal dominant inherited disease which is characterized by C1 inhibitor deficiency or dysfunction and recurrent attacks of angioedema. Angioedema most often seen in limbs, oropharynx and visceral organs. Intestinal mucosal edema can cause temporary bowel obstruction and severe abdominal pain that can be confused with acute abdomen. Abdominal attacks accompanied by 50% of all attacks. Before the diagnosis of hereditary angioedema some of the patients may undergo unnecessary appendectomy or exploratory laparotomy. The most feared complication of hereditary angioedema is laryngeal edema Case: Twelve years old boy presented with swelling of the face, eyes and limbs and recurrent abdominal pain since he was 1 year old. It was learned that the attacks resolve spontaneously within 48 hours and not accompanied by pruritus or urticaria and his uncle has similar complaints. When he was 9 years old, he has been appendectomy for abdominal pain. Physical examination was normal. C4:5,9 mg/dL (10-40) and C1 inhibitor: 8,1 mg/dL (2440) levels was found to be low. C1 inhibitor concentrate is prescribed for use in attacks. Conclusions: Hereditary angioedema is a rare autosomal dominant inherited disease which is characterized by C1 inhibitor deficiency or dysfunction and recurrent attacks of angioedema. C4 levels are low in almost all cases and it is used as a screening test. Corticosteroids, antihistamines and epinephrine which are used in the treatment of allergic angioedema has no place in the treatment of hereditary angioedema. To replace the missing C1 inhibitor for the treatment of attacks fresh frozen plasma and recombinant C1 inhibitor (CetorR) are used in our country. We want to raise awareness among emergency medicine, family and pediatric physicians about hereditary angioedema, a rare but potentially lethal disease, with this case report Keywords: Hereditary, Angioedema [P-101] A rare cause of cardiac arrest: catecholaminergic polymorphic ventricular tachycardia Firuze Erbek Alp1, Sezen Ugan Atik2, Reyhan Dedeoglu2, Aida Koka2, Funda Oztunc2, Ayse Guler Eroglu2 1 Istanbul University Cerrahpasa Medical Faculty Department of Pediatrics 2 Istanbul University Cerrahpasa Medical Faculty Pediatric Cardiology Department Introduction: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by episodic syncope occurring during exercise or acute emotional stress in children without structural cardiac abnormalities. In people with CPVT, these episodes typically begin in childhood. If CPVT is not recognized, an episode of ventricular tachycardia may lead to cardiac arrest and sudden death. Generally CPVT results from mutations in the RyR2 gene. Here we will present a case who was admitted to the emergency service with cardiac arrest and diagnosed as CPVT. Case: 2-year-old male patient presented to our emergency department with cardiac arrest. Cardiac arrest had developed after a syncope attack when he was playing. The patient had a history of recurrent syncope attacks and had been treated for epilepsy. It stated that the syncope usually occurred after exertion. Cardiopulmonary resuscitation (CPR) and intubation was performed and the patient was brought back to life. There were ventricular extrasystoles in the ECG. Because of hypotension dobutamine and norepinephrine treatment was started for positive inotropic support. After starting the dobutamine treatment ventricular tachycardia, ventricular fibrillation and cardiac arrest developed, respectively. Dobutamine was stopped immediately. Cardiopulmonary resuscitation was performed and the patient returned to sinus rhythm. Amiodarone was administered for ventricular tachycardia. Echocardiography couldn’t detect structural cardiac pathology. According to these findings, CPVT was suspected and genetic testing was performed. The genetic test revealed heterozygote missense mutation (c.9110G>A(p.Gly3037Asp)) in exon 64 of RYR2 gene. The patient did well under amiodaron treatment without any ventricular tachycardia. Intracardiac defibrillator implantation was recommended to patient’s family but they didn’t accepted because of severe neurological damage. Conclusion: Catecholaminergic polymorphic ventricular tachycardia is a rare cause of sudden cardiac death in children. Keywords: Catecholaminergic polymorphic ventricular tachycardia, cardiac arrest - 59 - [P-102] Prenatally diagnosed asymptomatic cardiac rhabdomyoma Firuze Erbek Alp1, Sezen Ugan Atik2, Aida Koka2, Reyhan Dedeoğlu2, Levent Saltık2 1 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatrics 2 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatric Cardiology Introduction: Rhabdomyoma is the most common cardiac tumor in children often associated with tuberous sclerosis (TS). Arrhythmia due to cardiac rhabdomyomas may be the initial sign of TS. We report a case of rhabdomyoma which diagnosed prenatally because of its large dimensions and despite of its large dimensions it was asymptomic during the postnatal course. Case: We presented a case of a preterm male newborn who was born to primigravida at 35 weeks of gestation. After birth the baby’s physical examination was unremarkable in the delivery room. The mass didn’t cause arrhythmias or inflow-outflow obstructions after birth. Fetal echocardiography at 31 weeks of gestation had revealed a large mass in the interventricular septum suggestive of rhabdomyoma. Postnatal echocardiogram demonstrated a large cardiac mass, which had large extensions (16x16 mm) along interventriculer septum. There was no evidence of inflow or outflow tract obstruction. No tachycardia was observed. There was no need for treatment. Renal ultrasonography, cranial Magnetic Resonance imaging, genetic evaluation for TS weren’t investigated yet. Family members were also examined for stigmas of TS and no significant clinical findings were detected. A month after discharge from the hospital, the child was doing well without any treatment. Follow-up echocardiographic assessments were planned. Our expectation was spontaneous regression of the mass in the following echocardiographic assessments. Discussion: While rhabdomyomas may be asymptomatic, they can also cause arrhythmias, inflow-outflow obstructions and even cardiac failure. The symptoms may occur depending on the anatomic location of the tumor. Keywords: cardiac rhabdomyoma, tuberous sclerosis [P-103] When the Paediatric Intensive Care Unit (ICU) plays the role of the Emergency Department (ED)... A two-year retrospective study Foteini Zafeiropoulou, Maria Vasilopoulou, Olga Giannouli, Zoi Beka, Leda Sianidou Paediatric Intensive Care Unit, Penteli Children’s Hospital, Athens, Greece Background & Aims: The role of an ED in a tertiary hospital is the emergency management and acute care of severely ill and injured patients. An ICU undertakes the continuous monitoring and specialized treatment of unstable and critical patients. In many settings, due to lack of organized EDs, patients in need of emergency treatment get admitted in the ICU and that is a burden to the patient as well as the health care system. This study aims to underscore the fact that a percentage of ICU admissions could have been managed in an organized ED. Methods: This is a retrospective observational study based on the ICU register during the period September 2013 until September 2015. Population: ICU admissions through the Outpatient Department. Inclusion criteria: a. Patients who received emergency treatment and got stable during the first few hours of their stay in the ICU. b. Patients who were nursed in the ICU solely for monitoring of their vital signs c. Patients who were admitted for interhospital transfer. Results: Total ICU admissions: 107. Twelve of those fulfilled the inclusion criteria representing 11,2% of the total. In particular, there were: a. 4 patients that were rapidly resuscitated after anaphylactic reaction, asthma crisis, croup and hypovolemia b. 5 that only needed vital signs’ monitoring and c. 3 stable patients with a new diagnosis of brain tumor that were transferred to neurosurgical centers. Conclusions: An ICU admission is an unpleasant experience for little patients. It comes with a number of tests and interventions and staying in an unhospitable environment without their family. Additionally, the daily cost of ICU stay climbs up to 700 €. It is self-evident that we ought to do everything in our power to decrease unnecessary ICU admissions. The development of organized EDs will be a key factor in this endeavor. Keywords: Emergency, Intensive, Paediatric [P-104] Protein loosing enteropathy in an infant with concomitant rotavirus infection and Celiac disease Gamze Ozgurhan1, Gokten Ongel1, Meltem Yildirim1, Lida Bulbul2, Zerrin Onal2, Bulent Hacihamdioglu1 1 Suleymaniye Maternity and Children’s Training and Research Hospital, Department of Pediatrics, Istanbul, Turkey 2 Bakirkoy Dr. Sadi Konuk Training and Research Hospital,Department of Pediatrics, Istanbul, Turkey Introduction: Protein-loosing enteropathy (PLE) is a rare complication of a variety of intestinal disorders characterized by an excessive loss of proteins into the gastrointestinal tract due to impaired integrity of the mucosa. The etiology of PLE is various. Rarely case of Rotavirus gastroenteritis complicated by PLE has been reported. It is known that Celiac disease is the reason of severe form of PLE. Here in we report an infant who was developed PLE after acute Rotavirus gastroenteritis and diagnosed as Celiac disease during follow-up. Case presentation: A 12-month-old boy presented with vomiting, diarrhea and weight loss for 7 day prior to admission. Subsequently, he was hospitalized - 60 - because of dehydration. The physical examinations revealed that weight: 8200 gr(weight for height: 81%), pretibial edema, and abdominal distention. Laboratory examinations showed that hypoalbuminemia (2.1 g/dl) with mild hypertransaminasemia. A stool examination was positive for Rotavirus antigen. There was not proteinuria in urinalyses. Abdominal ultrasound showed bowel expansion and intestinal wall thickening with a small amount of ascites. He diagnosed as Rotavirus infection induced PLE but in follow-up his diarrhea persisted more than 2 weeks. He was evaluated other reason of chronic diarrhea and antibodies associated with Celiac disease were detected positive. Intestinal biopsy was confirmed Celiac disease diagnosis. His symptoms were fully improved after gluten free diet. reproduction was seen in hemoculture and BOS culture, treatment was continued with cefotaxime. The patient was discharged with minimal-mid level hydrocephalus findings in cranial USG and MR following 21 days of antibiotics treatment. Conclusions: In clinical practice; if proteinuria is not detected in children admitted with edema, PLE must be considered. Rotavirus gastroenteritis is not always self-limited and may due to PLE especially infant with previous silent Celiac disease. Keywords: Protein-loosing enteropathy, Rotavirus, Celiac disease [P-106] [P-105] E.coli meningitis after rotavirus gastroenteritis in an infant Gamze Ozgurhan1, Oznur Vermezoglu1, Didem Ocal Topcu1, Adem Karbuz2, Bulent Hacihamdioglu1 1 Suleymaniye Maternity and Children’s Training and Research Hospital, Department of Pediatrics, Istanbul, Turkey. 2 Okmeydanı Training and Research Hospital, Department of Pediatrics, Istanbul, Turkey Introduction: Although Rotavirus gastroenteritis is quite common in pediatric population, secondary bacterial sepsis and/or meningitis following Rotavirus infection are rare clinical entities.Gram-negative bacilli are the fifth most common cause of meningitis in infants but its rarely occur after gastroenteritis. Here in we report an infant who developed E.coli meningitis after acute Rotavirus gastroenteritis. Case presentation: 2.5 month male infant with fever, vomiting and watery diarrhea that started 1 day ago was admitted to hospital. Past medical and family history revealed that he was born with term delivery with no special postnatal history. Apart from mild dehydration and irritation, physical examination was normal. Initial leukocyte count was 10.100/mm3 and C-reactive protein was 19,3 mg/L (references; 0-8). Rotavirus antigen in stool sample was positive. No pathogenic bacteria reproduction was seen in stool culture. He was hospitalized because of oral intolerance and dehydration due to Rotavirus gastroenteritis. Fever measured at second day of hospitalization was 39,5oC and he was irritable on physical examination. Lumbar puncture was done for suspicion of meningitis. In analysis of the CSF; a neutrophilic pleocytosis (68% PMNL, 32% lymphocytes), elevated protein (127 mg/dl), and reduced glucose concentration (11 mg/dl, simultaneous blood glucose: 91 mg/dl) were found. Intravenous vancomycin and cefotaxime were started empirically. Since E.coli Conclusions: Rotavirus infection is not always benign, pediatricians should be kept in mind bacterial translocations and severe life-threating infections in patients with Rotavirus infection.It is vital to start prompt antibiotic treatment as soon as the diagnosis of secondary bacterial infection is made because of high mortality and morbidity rates. Keywords: Rotavirus, bacterial sepsis 15-month case of septic arthritis without swelling and rash Gamze Sarıkaya Uzan1, Mustafa Uzan2, Funda Kökali Atash1, Bahar Çalışkan1, Nilgün Selçuk Duru1 1 Haseki Education and Research Hospital, Department of Pediatrics 2 Şişli Hamidiye Etfal Education and Research Hospital, Family Medicine C Introduction: Septic arthritis is the suppurative inflamation of the joint often caused by bacteria. Lifelong bone and joint damage affecting the childs life can develop due to inadequate diagnosis and delayed treatment. Clinical findings of the disease is mostly seen under age of 2 years and may be weak in this age group. Here we submit 15-month old septic arthritis case presented with fever but no redness or swelling of the knee joint. Case: 15-month-old girl, was admitted to emergency department with complaint of 38.4 ° C fever. At systemic examination, patient had poor general status and increased discomfort with pressure on non sweeling and non redness left knee, and no other findings. Blood samples showed that leukocyte: 19.1 10⁹/uL, C-Reactive Protein: 438mg/l (0-5). Arthrocentesis sample obtained from knee joint was cloudy, gray colur and high dansity and leukocyte: 150,000/mm ³ erythrocyte: 200/ mm ³ in cell counting. Orthopaedic clinicians took the patient to emergency operation. Vancomycin, ceftriaxone and age-appropriate rehydration therapy was started and postoperative 2. day acute phase reactants were significantly decreased. Synovial fluid cultures Staphylococcus aureus reproducing patient was discharged after 4 weeks of intravenous antibiotic treatment to child infection outpatient follow-up. Result: Fever is a symptom that has to be clarify the cause in children.Other symptoms and complaints accompanied with fever must be examined thoroughly and all systemic examination must be certainly done with detailed medical history. Even if they do not have any abnormality,locomotor system necessarily should be examined, joint sensitivity should be explored. This case is submitted to focus on detailed systemic examination especially on infants when fever cause culdnt be cleared, before invasive procedure and further screening. Keywords: Fever, Physical examination, Septic arthritis - 61 - [P-107] [P-108] Gizem Pamuk1, Fatih Varol2, Gürcan Dikme3, Hande Kumkumoğlu3, Duhan Demircioğlu1, Halit Çam2, Tülin Tiraje Celkan3 1 Istanbul University Cerrahpasa Medical Faculty, Department of Pediatrics, Istanbul 2 Istanbul University Cerrahpaşa Medical Faculty, Department of Pediatrics, Pediatric Emergency, Istanbul 3 Istanbul University Cerrahpaşa Medical Faculty, Department of Pediatrics, Pediatric HematologyOncology, Istanbul Introduction: Hemophilia A is an X-linked,recessive disorder caused by deficiency of functional plasma clotting factor VIII.The development of inhibitory antibodies to FVIII can result in acquired hemophilia A or can complicate the treatment of genetic cases.Patients with hemophilia A may bleed into tissues or from damaged skin and mucosal surfaces.Bleeding from the renal tract may not be recognized as a sign of a severe bleeding disorder in patients who have not undergone excretory urography.Awareness of other possible causes of hematuria should prompt more frequent investigation of the urinary tract in such patients.We report a case of spontaneous acute renal hematoma in a 5-year-old boy,who was diagnosed with hemophilia A. Gizem Pamuk1, Fatih Varol2, Duhan Demircioğlu1, Duygu Ülger1, Halit Çam2 1 Istanbul University Cerrahpasa Medical Faculty, Department of Pediatrics, Istanbul 2 Istanbul University Cerrahpaşa Medical Faculty, Department of Pediatrics, Pediatric Emergency, Istanbul Introduction: Cutis marmorata telangiectatica congenita is a congenital, rare, usually benign, sporadic, localized or generalized vascular lesion characterized by a persistent purple or blue “marbled” or “fishnet” appearance of the skin. In this report, we report a preterm female newborn with CMTC at birth who showed typical cutaneous features. Spontaneous renal hematoma in a patient with Hemophilia A Case: A 5-year-old boy diagnosed with hemophilia A was presented with painless gross haematuria that had started 5 days before admission.There was no history of trauma or fever.At the time of presentation of hematuria,he was prescribed metilprednisolone.The hematuria persisted on the 5th day of metilprednisolone and he was referred to the pediatric emergency clinic. At the admission,vital signs revealed blood pressure 110/70 mmHg,respiratory rate 23/min,pulse rate 96/min and body temperature 36.7°C.The laboratory test results were as follows:haematocrit 37,6%,haemoglobin 11,9 g/ dl,white blood cell 11,5×103/μl,platelet 792×103/ μl,prothrombin time 11,4 s, partial thromboplastin time 84,8 s,INR:0,9, serum albumin 4,2 g/dl, serum urea 22 mg/dl and creatinine 0,5 mg/dl.Urinalysis showed many red blood cells without pyuria.Urine culture was negative. Kidney ureter bladder radiography did not reveal any radio opaque stone, while the initial abdominal US imaging revealed a hyperechogenic image of 14x12 mm in the left renal pelvis.The doppler US revealed no abnormality.Bed rest and iv hidration treatment was ordered. He responded well to treatment: the haematuria has gradually regressed. Conclusion: Renal hematoma should be considered as one of the possible causes of haematuria in hemofilia patients.Awarness of this cause should prompt more frequent investigation of the urinary tract in such patients. Keywords: Hemophilia A, hematuria, renal hematoma Cutis Marmorata Telangiectatica Congenita in a female newborn: A Case Report Case: A female newborn was presented with persistent blue marbled appearance of the skin since birth. She was the first child of non-related parents, spontaneously delivered at 33 weeks of gestational age. During first hours of life, she was interned in a neonatal intensive care unit due to respiratory distress with the diagnosis of transient tachypnea of the newborn and discharged after 20 days. Since birth marbled bluish and deep purple reticulated skin lesions involving the whole body were noted. Physical examination showed reticulated bluish purple skin changes all over the body including the face and limb. The lesions were unvarying and permanent and became more visible with crying and exposure to room temperature. The baby had normal facies, with head circumference of 33 cm and birth weight 2135 gr, without other vascular anomalies or asymmetry of the growth of the limbs. The systemic examination was normal.The laboratory test showed haematocrit 29,4%,haemoglobin 9,8 g/dl,white blood cell 10,5×103/μl,platelet 410×103/ μl,serum urea 5 mg/dl and creatinine 0,4 mg/dl,serum albumin 3,6 g/dl. Serum electrolytes, liver enzyme tests and serum lactate level were normal. The abdominal and cranial ultrasound imaging were normal. Conclusion: Clinical characteristics and persistence of cutis marmorata prompted the diagnosis of Cutis Marmorata Telangiectatica Congenita.Vascular anomalies as Sturge–Weber syndrome, Klippel–Trenaunay–Weber syndrome have been associated with CMTC. The case we reporte had a normal face with no other vascular abnormality, and systemic examination was normal. She had no asymmetry of the limbs.The disorder is self limiting and treatment is not necessary unless complicated with other associated anomalies. Keywords: Cutis marmorata telangiectatica congenita, cutis marmorata, congenital vascular anomaly - 62 - [P-109] [P-110] Diagnosis of Pseudohypoparathyroidism during a routine care of an asthmatic child: Do pediatricians aware enough about the disease? Gaucher disease in Russia - pediatric experience Gizem Sayar Bilgin1, Zehra Yavaş Abalı2, Muhammet Ali Varkal1, İsmail Yıldız1, Firdevs Baş2, Emin Ünüvar1, Ayşe Kılıç1 1 Istanbul University Istanbul Medical Faculty, Pediatrics, General Pediatrics, Istanbul 2 Istanbul University Istanbul Medical Faculty, Pediatrics, Pediattric Endocrinology, Istanbul Background: Pseudohypoparathyroidism (PHP) is characterized by hypocalcemia, hyperphosphatemia, increased serum concentration of parathyroid hormone (PTH), and insensitivity to the biologic activity of PTH. We report a patient diagnosed with PHP incidentally during her routine control. Case: A seven year old girl presented with the history of recurrent cough since she was one year of age. Two years ago before the admission she had been incidentally diagnosed as having mild low calcium values and she had been treated with oral vitamin D. Vitamin D and serum calcium levels were normal within 3 months of treatment. The family didn’t took her to regular visits for about two years. On her recent admission she evaluated for spasms in her face. Her weight 30.6 kg (1.71 SDS), height 135 cm (2.80 SDS), and body mass index were 16.8 kg/m2 (0.6 SDS). Chvostec’s sign was positive. Systemic examinations were normal and there were no subcutaneous calcifications. Besides mild round face, she had no AHO feature and no intellectual disability. At first evaluation serum calcium:6.5 mg/ dl (8.8-10.2 mg/dl), phosphorus: 7.68 mg/dl (2.7-4.5 mg/dl), magnesium:1.77mg/dl (1.5-2.5 mg/dl) alkaline phosphatase:421U/l (70-300 U/l), 25 hydroxy vitamin D3:35.5 ng/ml (>20 ng/ml) and PTH were 1400pg/ml (15-65 pg/ml). There were no renal pathologies and she had hypocalciuria. After the treatment of symptomatic hypocalcemia, oral calcium replacement and calcitriol were added to the therapy. Symptoms resolved and serum calcium gradually increased. Her 4th metacarpals were short and brain MRI revealed no calcification. Conclusion: In this patient with PHP we learned that as a rare disease PHP must come to attention by pediatricians when evaluating children with hypocalcemia. As it’s already known, hypocalcemia may induce laryngospasm and cough/respiratory problems in this patient’s history may also misdiagnose hypocalcemic episodes. Keywords: asymptomatic, Pseudohypoparathyroidism Goar Movsisyan, Leyla Namazova Baranova, Kirill Savost’anov, Olga Gundobina FSBI “Scientific Center of Children’s Health” of the Ministry of Health of the Russian Federation Background&Aims: To describe our 15 years experience in the management of patients with Gaucher disease(GD) by multidisciplinary assessment. Methods: An observational, retro- and prospective study based on Russian pediatric GD registry data of SCСH. The diagnosis was confirmed by biochemical and molecular genetic research. Results: Based on registry data 2000-2015 years the total number of pediatric patients with GD in Russia was 90; the age was between 0 to 18 years; M:F 39:51; Type1:Type2:Type3 78(87%):2(2%):10(11%). The mean age of a debut and diagnosis was less then 7 years. The most common signs and symptoms noted were: growth retardation (25% in or below the third percentile for weight and 34% in height; majority between ages of 2 to 5 years), hepatosplenomegaly (95%, 4 patients were splenectomized), anemia(90%), thrombocytopenia(85%), skeletal involvement by X-ray(85%), bone pain(30%), bone crises(10%), hemorrhagic syndrome(65%), reduced bone mineral density(60%), neurologic symptoms (11%). All patients had low glucocerebrosidase activity. In molecular analysis we identified 13 novel mutations; genotypes N370S/L444P and N370S/W184R were most common in type1, the most common genotype identified in neuronopathic forms was L444P/L444P. The additional important biochemical tests at diagnosis was high chitotriosidase activity and ferritin level; low iron, serum lipids and v.B12 levels. The 87 patients were treated by imiglucerase, 3 patients - by velaglucerase alpha, the dose of ERT in type1 was from 30 to 60 U/kg/2weeks, in type3 - from 60 to 100 U/kg/2weeks. Most of our patients presented the significant improvement of the course of disease after 2-10 years of ERT. Conclusions: Our study shows the wide clinical and genetic variability of GD in pediatric patients. National pediatric GD registry is a good model for other rare diseases because it provides us the advanced knowledge about certain pathology and helps to improve the management and the life quality of these patients. Keywords: Gaucher disease, multidisciplinary assessment, children - 63 - [P-111] [P-112] Leukocyte Adhesion Deficiency Gökçen Kamış1, Esin Aldemir2, Çiğdem Aydoğmuş3, Sultan Kavuncuoğlu2 1 Kanuni Sultan Süleyman Training and Research Hospital,Department of Pediatrics 2 Kanuni Sultan Süleyman Training and Research Hospital,Neonatology Clinics 3 Kanuni Sultan Süleyman Training and Research Hospital,Pediatric İmmunology Clinics Introduction: Leukocyte adhesion deficiency(LAD) is a rare primary immune deficiency that characterized by inability of neutrophil migration to inflammatory area. In our clinics, an infant who referred to the hospital with malaise and high grade fever is diagnosed with LAD-1 severe form after further investigations.Because of the rarity of this syndrome,the case is presented. Case Presentation: A four days old male referred to the our clinics with high grade fever, feeding difficulty and grunting. Laboratory investigations showed WBC:34x103/µL,ANC:27x103/µL,CRP:115 mg/L. Chest radiography was normal. After blood, urine and CSF culturing, the antibiotic treatment was started as Ampicilina, Cefotaxime and Amikasin. Blood and CSF culture result in growth of Gram(+) alpha hemolytic streptococcus which is susceptible to the treatment. During hospitalization, fever relapsed and he has omphalitis and nonpurulent abscess formation in axillary skin area. His laboratory findings showed WBC:36x103/ µL, ANC:30x103/µL, CRP:36,2mg/L, Procalcitonin:0,17. Vancomycin and Cefoperazone+Sulbactam is started. Blood immunoglobulin levels were found in normal by age. In the day 26,his umbilical cord detached.Flow cytometry shows the leukocyte CD18 levels of %0.He was diagnosed with LAD-1 severe form. Discussion: Migration of leukocytes from circulation to inflammatory area has four steps:recruitment,rolling,firm adhesion,extravasation. In LAD-1,leukocytes are unable to perform firm adhesion to the blood vessel walls due to the deficiency of ß2 integrin subunit,CD18. This molecule is coded by ITGB2 gene,chromosome 21q22.3.Thus far 86 different mutations have been defined in the gene The levels of CD18 determine the severity. CD18 expression is below %2 in severe form. Immune deficiency is seen in early infancy. Th symptoms are delayed umbilical cord detachment,omphalitis,recurrent and resistant infections,nonpurulent necrotizing infections Laboratory findings are remarkable neutrophilia,leukocytosis,high inflammatory markers. Conclusions: In the cases of severe neonatal sepsis that has low response to antibiotics, severe leukocytosis with neutrophilia,recurrent nonpurulan infections and the delay of the umbilical cord detachment,LAD-1 should be considered Keywords: immunodeficiency, infant Coexistence of Glanzmann’s thrombastenia and Glucose 6 Phosphate Dehydrogenase deficiency in one patient Gözde Yücel1, Gürcan Dikme2, Hande Kızılocak Kumkumoğlu2, Begüm Şirin Koç2, Gül Nihal Özdemir2, Tülin Tiraje Celkan2 1 Istanbul University, Cerrahpaşa Medical Faculty, Department of Pediatrics 2 Istanbul University, Cerrahpaşa Medical Faculty, Department of Pediatric Hematology Oncology Introduction: Glanzmann’s thrombastenia (GT) is a genetic platelet surface receptor disorder of GPIIb/ IIIa which results in faulty platelet aggregation, diminished clot retraction and it is more common in ethnic groups that display higher rates of consanguinity. Case: A 3 month old male patient was brought into our emergency department with a widespread rash of one week duration. The patient have parents with consanguinity and a birth history of a term C/S delivery without any significant complication. Postnatally he had prolonged jaundice and after the etiologic assesment, at 1 month of age, he was diagnosed with Glucose 6 Phosphate Dehydrogenase deficiency with an enzyme level of 6mU/10 RBC. The first physical examination revealed a petechial rash that is widely distributed over the face; the whole body of the child with many scattered purpuric lesions. According to the parents the occurrence of these lesions was spontaneous and unprovoked. There was not any associated abnormal physical findings. The complete blood count was entirely normal: Hemoglobin level 9.7 g/dL, white blood cell count 10400/mm3, platelet count 350000/mm3. The PT (12.4 seconds) and aPTT (30.6 seconds) were also normal. However, the bleeding time was prolonged; more than 10 minutes, which warranted further investigation. For assessing platelet function we used light transmission aggregometry (LTA); platelet aggregation failed to occur with ADP and collagen, except ristocetin, where the reaction was found to be preserved. For further analysis we used flow cytometry. This analysis revealed that CD41(GPIIb) and CD61 (GPIIIa) levels were absent. Overall, our patient with G6PD deficiency was also diagnosed with Glanzmann’s thrombastenia. Conclusion: We have diagnosed 2 distinct hematologic entities in one patient which are not related and have not been observed coincidentally in our clinical practice before. Keywords: Glanzmann’s thrombastenia, glucose 6 phosphate dehydrogenase deficiency, purpura - 64 - [P-113] Cayler Cardiofacial Syndrome: A rare condition in newborns Gunce Basarir1, Bedia Deniz Ekici1, Abdülhamit Tüten2, Handan Hakyemez Toptan2, Nilgün Karadağ2, Gülsen Akay1, Güner Karatekin2 1 Children’s Health Department, Zeynep Kamil Gynecologic and Pediatric Training and Research Hospital, Istanbul, Turkey 2 Newborn Intensive Care Unit, Zeynep Kamil Gynecologic and Pediatric Training and Research Hospital, Istanbul, Turkey Cayler cardiofacial syndrome is a rare syndrome characterised by asymmetric crying facies associated with unilateral absence or hypoplasia of the depressor anguli oris muscle, and congenital heart defects. Asymmetric crying facies (ACF) is usually noticed when a newborn cries and the affected angle of the mouth is pulled downward while contralateral side is immobile. The face seems symmetric during sleep. ACF can be seen as an isolated minor event but the important aspect of this abnormality is the association with other congenital anomalies; mostly in the cardiovascular system. Coexistance of unilateral hypoplasia/aplasia of the depressor anguli oris muscle and various congenital heart defects is called Cayler snyndrome and we report a case of ACF with tricuspid atresia, ventricular septal defect (perimembranous), atrial septal defect (secundum), patent foramen ovale, and proximal stenosis of left pulmonary artery. We emphasize the importance of being aware that this minor facial anomaly may be associated with other congenital anomalies; cardiac defects mostly. Keywords: Cayler, cardiofacial [P-114] Late Diagnosis of DHPR Deficiency Treated as PKU: A Case Report Gurkan Tarcin1, Ertugrul Kiykim2, Tanyel Zubarioglu2, Ayse Cigdem Aktuglu Zeybek2, Erdogan Soyucen3, Serif Cansever4 1 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatrics, Istanbul, TURKEY 2 Istanbul University Cerrahpasa Medical Faculty, Department of Pediatrics Division of Nutrition and Metabolism, Istanbul, TURKEY 3 Akdeniz University, Department of Pediatrics Division of Nutrition and Metabolism, Antalya, TURKEY 4 Istanbul University, Cerrahpasa Medical Faculty Central Laboratory, Istanbul, TURKEY Dihydropteridinereductase (DHPR) deficiency is an inborn defect of tetrahydrobiopterin (BH4) recycling that leads to decrease in levels of dopamine, serotonin and catecholamines in cerebrospinal fluid (CSF) and causes neurological symptoms such as hypotonia, psychomotor delay and seizures. Here, we present a case of a 27-monthsold male patient who is lately diagnosed as DHPR deficiency after having been followed up with a misdiagnosis of phenylketonuria (PKU) since 2 months of age. A male infant was diagnosed as mild PKU according to the results of newborn screening and was begun to receive phenylalanine (phe) restricted diet. Phe levels were monitored closely and found between normal ranges. At 6 months of age, generalized hypotonia developed and phe levels started to increase. Patient was referred to our outpatient clinic for further evaluation and treatment. His physical examination revealed profound truncal hypotonia dominated with dystonic movement disorder. BH4 metabolism disorders were considered in differential diagnosis due to hyperphenilalaninemia and neurological findings. Neurotransmitter levels and pterin metabolites were performed in CSF sample and found compatible with DHPR deficiency. L-Dopa, 5-hydroxytryptophan and folinic acid replacement therapies and phe restricted diet were started immediately and the patient achieved a good clinical response. In our country, frequency of inherited metabolic diseases is high due to consanguineous marriages. As a result, all neonates should be screened for metabolic disorders. In all patients who have hyperphenylalaninemia in metabolic investigations, BH4 metabolism disorders should be included in differential diagnosis. Keywords: Dihydropteridinereductase deficiency, DHPR deficiency [P-115] Osteogenesis imperfecta – experience of Scientific Center of Children’s Health in Moscow Guzal Yakhyaeva, Tea Margieva, Kirill Savostyanov, Alexander Pushkov, Natalia Jurkova, Konstantin Jerdev, Nato Vashakmadze, Anait Gevorkyan, Alexander Baranov, Leyla Namazova Baranova Scientific Center of Children’s Health Background&Aims: Osteogenesis imperfecta (OI) is a hereditary genetic disorder of connective tissue, characterized by bone fragility and recurrent fractures due to the defects of collagen 1 type. We want to describe our 4 year experience in the management of patients with OI by multidisciplinary assessment. Methods: An observational, retrospective study based on data obtained from the analysis of the clinical files of all patients with OI. The studied variables were: age at diagnosis, familial history of OI, age at fracture, number of fractures, molecular genetic mutation (by nextgeneration sequencing (NGS), medical/surgical therapy Results: 30 pediatric patients (male=19; female=11, mean age 6.1 years), by clinical and radiographic evaluation registered n=12 type I, n=9 type III, n=8 type IV, n=1 type V. 8 patients had familial history of OI. A causative variant in the COL1A1 gene was found in 5 cases; in the COL1A2 gene was found in 3 cases. A causative variant in the COL1A1 gene near the amino (N)-proteinase cleavage site, which result an overlap OI/ Ehlers-Danlos syndrome was found in 1 case. In other 7 cases was found mutations in the COL5A1/2 gene (n=5), in the COL3A1 gene (n=1). 19 patients underwent medical treatment with pamidronate, starting at an average of 4.16 years. In follow-up sample (n=18) there was a decrease in the number of fractures after starting treatment (average 9.1 to 5.8 fractures/patient). - 65 - Surgical treatment of bone deformities was performed in 10 patients (33,3%). Conclusions: OI is disease with a wide clinical and genetic variability with insignificant molecular-genetic correlation. There are still no effective treatment, however medical treatment with early intervention of bisphosphonates seems to reduce the incidence of new fractures and surgical treatment with intramedullary rods, the last ones not registered in Russia, seems to improve functional outcomes and decrease the level of disability Keywords: bone fragility, collagen 1 type, pamidronate [P-116] two brothers who were diagnosed with hyper IgE syndrome on the basis of Molluscum contagiosum Mebrure Yazıcı1, Ayşegül Demir2, Gülsüm Güzel2, Mustafa Atilla Nursoy1, Erkan Çakır3, Özlem Su4 1 Bezmialem Vakıf üniversitesi Çocuk Alerji Bilim Dalı 2 Bezmialem Vakıf üniversitesi Çocuk Sağlığı ve Hastalıkları Bilim Dalı 3 Bezmialem Vakıf üniversitesi Çocuk Göğüs Hastalıkları Bilim Dalı 4 Bezmialem Vakıf üniversitesi Dermatoloji Bilim Dalı Introduction: OD hyper-IgE which is a type of eczema, that is usually observed in recurrent skin and lung infections with high serum IgE levels on various skeletal systems, which is a rare primary immune deficiency syndrome associated with connective tissue and vascular anomalies. Hyper IgE syndrome is an autosomal recessive severe eczema type with recurrent bacterial and viral skin infections (such as Molluscum contagiosum) which could be observed with neurological symptoms in addition to sinopulmonary infection. Molluscum contagiosum is a round and saddle-backed DNA pox virus infection characterized by white papules, which is commonly observed on the facial skin, the eyelids, the neck and axilla. Case: A 9 and a 3 years-old two brothers who are recurrent lung infections, was admitted to our clinic with complaints of cough and phlegm. The older brother had the recurrent lung infections while his younger brother since 4 months had similar infections and it was found out that on their body along with these infections contain wart-like lesions. During the whole body examination many saddle-backed (middle sunken)white papules were detected. Molluscum contagiosum infection was diagnosed with a skin biopsy. We have noticed that there was also the big brother’s finger clubbing. We have also observed on big brother’s chest CT lesions and bilateral infiltrates with bronchiectasis after seeing chronic changes and problems on chest X-ray. Lymphocyte subsets, IgA, IgG and IgM levels were within normal limits while it was thought there is an immune deficiency during the investigation of the patients. Younger brother’s IgE levels was found to be 530 IU / mL while the older one’s was 2779 IU / ml. After these findings the patients were diagnosed with hyper IgE syndrome on the basis of Molluscum contagiosum. infiltrations can be seen in hyper-IgE syndrome. Keywords: Molluscum contagiosum, OD hyper-IgE [P-117] A Rare Infection in Children: Pelvic Osteomyelitis Halil Özdemir1, Tuğçe Tural Kara1, Adem Karbuz2, Anıl Tapısız3, Suat Fitöz4, Ergin Çiftçi1, Erdal İnce1 1 Department of Pediatric Infectious Diseases, Ankara University Medical School, Ankara, Turkey 2 Department of Pediatric Infectious Diseases, Okmeydani Education and Research Hospital, İstanbul, Turkey 3 Department of Pediatric Infectious Diseases, Gazi University Medical School, Ankara, Turkey 4 Department of Pediatric Radiology, Ankara University Medical School, Ankara, Turkey Acute hematogenous osteomyelitis is typically seen in long tubular bones in children. Pelvic bone involvement is so rare and diagnosis is sometimes delayed, due to deep localization of infection. Herein, we report a pediatric patient with pelvic osteomyelitis. A 15-year-old girl was admitted because of the left groin pain without trauma and fever. The pain was continuing approximately one month which was constant during rest and movement. On physical examination, temperature was 38.0 C⁰. She could not walk without support. Besides she did not exactly stand on his left foot. Her hip joint was limited in movement. Pelvic radiography did not show any pathological findings. Magnetic resonance imaging showed bone marrow and muscles were detected with pathological signal changes during ischium-pubic branch. We considered osteomyelitis and malignant infiltrative process. Then, bone marrow aspiration was done and it was found to be normal. Bone scintigraphy determined symphysis pubic, lower part of the left sacroiliac joint and left acetabular region had increased uptake activity, left iskium bone revealed pathological uptake. Biopsy and curetage of infiltrative lesion was performed. Biopsy material culture was unremarkable. Tuberculosis polymerase chain reaction, acid resistant bacillus and culture were negative, but the diagnosis of osteomyelitis was confirmed by pathology results. Ceftriaxone treatment was given during one month. Also oral ciprofloxacin and amoxicillin-clavulanate treatment was received one month after discharge. At the end of treatment the patient recovered without sequelae. As a result; pelvic osteomyelitis is a rare infection in children. Pelvic magnetic resonance and scintigraphy are useful for diagnosis. However, diagnoses should be confirmed with culture. Sometimes diagnosis delays due to deep localization of site infection. If the diagnosis is suspected, imaging techniques should be used. Keywords: Acute hematogenous osteomyelitis, children, pelvic osteomyelitis Conclusion: Moloscum contagiosum and chronic lung - 66 - [P-118] SPINAL TUBERCULOSIS (POTT’S DISEASE), A CASE REPORT Halise Zeynep Işcan1, Özgür Genç2, Özgecan Avcı1 1 Department of Pediatrics,Şişli Hamidiye Etfal Training and Research Hospital,İstanbul,Turkey 2 Departmant of Radiology,Şişli Hamidiye Etfal Training and Research Hospital,İstanbul,Turkey Aim: Tuberculosis is primarily a pulmonary infection,extrapulmonary manifestations are not uncommon,especially in children. The classic manifestation of tuberculous spondylitis is progression to Pott’s disease,in which destruction of the vertebral bodies.Early diagnosis and treatment is important to avoid complications.We hereby present a case getting spinal tuberculosis diagnosis which starts with a complaint of the incapability to walk at the age of 2. Case: A 2-year-old female patient had abrupt complaint of incapability to walk,back pain.Thoracic intradural mass was observed in spinal MR.By external centre,a biopsy was conducted on it,resulted necrotizing granulomatous inflammation.In physical examination of the patient who was sent to us,there was plegia in both lower extremities.Gastric fluid was taken 3 times,it was ARB negative and there was no reproduction in culture results.PPD was negative(8mm,there is one BCG),she had a bidirectional chest scan,it was normal. Her family scan was negative,Quantiferon was negative. In spinal MR that she had before treatment, nodular lesion covering total L2 corpus vertebrae and a lesion including cystic expansion and septation covering in spinal tract between D8-L2 were detected. 4(quad) anti-tuberculosis treatment was started.Prednol(2mg/ kg/day) was started for pressure findings.Her left leg movements improved on 4th day of prednol.Prednol treatment was in exact dose during 4 weeks,in halfdose during 2 weeks,stopped at the end of 6 weeks. After 2 months,treatment is changed to 2(dual) antituberculosis.She still receives 2(dual) anti-tuberculosis treatment (3rd month of treatment),is followed by Paediatric Infectious Disease Clinic. She does physical treatment exercises and can put her feet on the ground. Conclusion: Spinal tuberculosis(Pott’s Disease) is a disease remaining to be diagnosed as its onset is insidious,its radiological findings progress slowly.It has early and late neurological findings,even if early neurological findings go down by anti-tuberculosis treatment, late neurological cases are mostly irreversible. Prognosis is excellent with adequate medical treatment. Keywords: Tuberculosis, Pott’s Disease, Neurological Deficit [P-119] evaluation of nosocomial bloodstream infections in our unit detected in a 3-year period Adil Umut Zübarioğlu, Ali Bülbül, Halise Zeynep Işcan, Hasan Sinan Uslu, Evrim Kıray Baş, Duygu Besnili Acar Department of Pediatrics,Şişli Hamidiye Etfal Training and Research Hospital,İstanbul,Turkey Introduction: Nosocomial blood stream infection is an important cause of mortality and morbidity in neonatal intensive care units (NICU). In this study, it was aimed to determine blood culture positive nosocomial infections in 3 year period(2011-2013). Materials-Methods: Blood culture samples, taken from the NBCU between 2011-2013, were examined. Reproduction was determined.Organisms, isolated from blood culture, association with the length of stay, gestational age and birth weight of the newborn, antibiotic susceptibility,mortality rates were determined. Findings: Throughout the study period,121 blood culture positive nosocomial infection episodes in 85 cases were evaluated. 55(64.7%)of the cases was premature,38 (44.7%)was female,mean gestational age was 31 weeks,mean birth weight was 1812gr.The most common admission diagnoses were prematurity(n=55, 64.7%), congenital anomaly/syndromic infants(n=10, 11.8%),perinatal asphyxia(n=7, 8.2%).Mean hospitalization duration was 67 days.Distribution of the microorganisms; gram positive bacteria 65.3%(n=79), gram negative bacteria 26.4%(n=32),candida 8.3% (n=10).While coagulase-negative staphylococci(n=69) was seen most among gram positive bacteria, klebsiella species of gram negative agents(n=12) were most common. Number of the cases lost in the study group was 17 (premature; n=10, congenital anomalies / syndromic infants; n=5, asphyxia; n=2) and mortality rate was 20%.The gestational weeks of the newborns who were lost were significantly(p=0.025). Antibiotic resistance of the gram positive factors were as follows; penicillin 100%, clindamycin 63.3%, methicillin 89.8% and vancomycin resistance 26.6%. As to gram negative bacteria; gentamicin 65.6%, cefotaxime 78%, amikacin 28% and carbapenems 18.7%. Result: In our study, it was revealed that in our NICU, nosocomial infections occurred mostly due to gram positive factors, mortality in nosocomial infections was higher in those with low birth weeks, and antibiotic resistance was high in all nosocomial infections. Keywords: nosocomial bloodstream infections, neonatal intensive care units [P-120] Hyperımmunglobulin E Syndrome: A Case Report Abdulkadir Bozaykut, Handan Ayhan Akoğlu, Rabia Gönül Sezer, Nil Yazar Alpay Zeynep Kamil Maternity and Childrens’ Diseases Training and Research Hospital, Istanbul,Turkey Introduction: Hyperimmunoglobulin E syndrome (HIE) is a primary immunodeficiency disease characterized by markedly high titers of serum immunoglobulin E, chronic eczema, recurrent staphylococcal infections, pneumatoceles, reduced neutrophil chemotaxis and variable impaired T cell function. The main characteristic laboratory abnormalities are highly elevated serum IgE levels and eosinophilia. Here, we report a case with hyperimmunglobulin E syndrome. - 67 - Case: A 45 days-old male infant presented with fever,vomiting and swelling in left underarm, hospitalized with the diagnosis of lymphadenitis. He was the first child of a non-consanguineous marriage. He had a history of hospitalization in the newborn period because of hypoglycemia, ichthyosis, leukocytosis and sepsis with MRSA and candida in blood cultures. On examination he had coarse facial features, rough skin (Figure 1 and 2), palpable axillary and inguinal lymph nodes, other system examinations were normal. Laboratory investigations revealed; Hg 7.5 g/dl, WBC 28900/mm3, absolute eosinophil count 550/mm3, platelet 74000/mm3, CRP 5.2 mg/dl, serum IgE 978 IU/ ml (range:0-15 IU/ml). Bone marrow aspiration revealed eosinophilia and increased eosinophil precursor cells with no malignant infiltration. Hyperimmunoglobulin E syndrome was suspected and further investigation was scheduled but unfortunately patient was lost due to sudden clinical deteriation with septic shock. Conclusion: Hyperimmunoglobulin E syndrome is a rare genetic disease characterized with coarse facies, recurring cutaneous and visceral bacterial infections due to staphylococci, severe generalized eczema, which may be associated with osteoporosis. Biochemistry includes a high level of total and specific IgE and blood eosinophilia. Although the etiology of HIE syndrome is unknown, there is evidence that patients with this syndrome have abnormalities in cellular and humoral immune responses. We present this case to remind that an abcess could present first as a lympadenitis and in cases of suspected immundeficiencies, prompt antibiotic treatment can be life-saving. Keywords: Hyperımmunglobilin E Syndrome, Eozinofilia, Leukocytosis [P-121] Does a child need to go to a routine dental visit: parents’view Rabia Gönül Sezer, Abdulkadir Bozaykut, Handan Ayhan Akoğlu Zeynep Kamil Maternity and Childrens’ Diseases Training and Research Hospital, Istanbul,Turkey Aim: In Turkey, 84.9 % of children aged between 5-9 have dental caries which is an important health issue. We aim to evaluate the views and knowledge of parents about the need and the timing for the first dental visit of their children. Methods: Parents from pediatrics outpatient clinics were invited to fill in a questionnaire. The survey included questions on socio-demographic characteristics; parents gender, age, number of children, parents education level, opinion on the need for routine dental visits and the age at which a child should attend the first dental visit. The statistical analyses were made by SPSS version 17. Results: Two hundred sixty-nine questionnaires were returned Mean age of the parents was 30.6±6.9 years (range: 18-62, median: 30). The participants had amedian of 2 children (range: 1-8). The study population consisted of 231 (85.9 %) mothers and 38 (14.1 %) fathers and 33 (12.3 %) parents had a university degree. Only 45 parents answered both of the two questions correctly. 195 (72.5 %) of parents agreed that a child should go routinely to a dentist and only 60 (22.3 %) suggested the age for the first dental visit to be under 1-years of age. There was no significant relationship between the gender of the parents, number of children, educational status, age of the parents and the correct answers (p>0.05). Conclusion: Childrens’ oral health has a serious impact on childrens’ general health. Parents knowledge and attitudes about dental care are important issues for the improvement of children’s oral health. Educational public health programs may help improve the knowledge about dental health, also pediatricians unique role in child care should include parental education about dental care. Keywords: dental care, children [P-122] Two siblings with congenital glucose galactose malabsorption from the same family Hasret Ayyildiz Civan1, Tanyel Zubarioglu2, Ayse Cigdem Aktuglu Zeybek2, Yair Anikster3, Ben Pode Shakked3, Tufan Kutlu1 1 Istanbul University Cerrahpasa Medicine Faculty Department of Pediatric Gastroenterology, Hepatology and Nutrition 2 Istanbul University Cerrahpasa Medicine Faculty Department of Pediatric Metabolism and Nutrition 3 Tel Aviv University Faculty of Medicine Department of Medical Genetics Background: Congenital glucose galactose malabsorption (cGGM) is a rare disease with an autosomal recessive pattern of inheritance. The disorder leading to osmotic diarrhea in early infancy is caused by mutations in the Na+/glucose cotransporter gene SLC5A1 which encodes SGLT1 protein, a sodium dependent transporter, providing absorption of glucose and galactose from the brush borders of small intestine. Case Report: In this report we present two siblings with tenacious diarrhea from the same family whose parent had a second degree of kinship. Our first case was a two-month-old male infant admitted with fever, growth retardation, nephrolithiasis, acute renal failure, hypoalbuminemia and diarrhea. As he did not recover from renal failure with medical treatment, hemodialysis was initiated. By the fourth day of treatment, he had normal levels of creatinine and electrolytes. However, diarrhea was present since his birth and continued. After ruling out microbiological and biochemical causes, breastfeeding was quitted and a formula containing fructose as the main carbohydrate source was initiated for a possible diagnosis of cGGM. After the formula, frequency of defecation decreased immediately and other biochemical markers improved. Our second case was the sister of the previous patient. She admitted with diarrhea and growth retardation when she was 7-monthold. As her elder brother was diagnosed with cGGM and the current symtoms were mild form of that he suffered, cGGM was considered as a possible diagnosis and - 68 - feeding with a fructose-based formula was tried. It was also efficacious for her symptoms. Both of them gained weight following the treatment and an homozygous mutation of SLC5A1 gene was detected in their gene analysis. Conclusion: As the disease can progress with lethal complications owing to lack of early diagnosis, it should be included in differential diagnosis of patients with chronic diarrhea in early infancy. Diet is useful for both diagnosis and treatment. Keywords: infantile diarrhea, malabsorption, SLC5A1 [P-123] Corrosive esophagitis in a two days old neonate Hasret Ayyildiz Civan1, Sahin Hamilcikan2, Gulnaz Sariyeva1, Didem Gulcu3, Tulay Erkan1 1 Istanbul University Cerrahpasa Medicine Faculty Department of Pediatric Gastroenterology, Hepatology and Nutrition 2 Istanbul Sisli Kolan Hospital, Neonatal Intensive Care Unit 3 Adana Gynecologic, Obstetric and Paediatric Diseases Hospital, Department of Paediatric Gastroenterology Background: Benzalkonium chloride is a caustic agent which is used in farms, homes and hospitals for cleaning skin, mucous membranes and wounds as an antiseptic solution. Caustic substances may lead to respiratory and/or digestive system injuries in case of ingestion. Such injuries are rare in neonates and emanate mostly from erroneous practices of parents. Case Report: We present a two-days-old newborn case which was carried to the emergency unit with complaints of poor breastfeeding, uneasiness and crying for 4-6 hours. Her physical examination was unremarkable except mildly elevated intestinal sounds and weakness of sucking reflex. When her mom was questioned again, it was revealed that she had given a spoon of 10% BAC solution (approx.10-15cc) for her cough with the intention of treatment and that she did not vomit thereafter. Blood gases and laboratory blood tests were in normal ranges. A gastroscopy performed in the second hour of her admission revealed an hyperemic and edematous mucosa in the middle third of esophagus and a circumferential mucosal ulceration followed in the distal portion. She was transferred to neonatal intensive care unit and a conservative treatment including intravenous fluid, total parenteral nutrition, H2 receptor blocker and an antibiotherapy of cephasol and amicasin for 10 days was administered. A control gastroscopy on the 10th day of hospitalization demonstrated that the damage on the mucosa was almost totally improved. She was the youngest case with this etiology and successfully treated with conservative approach. Keywords: corrosive, benzalkonium chloride, neonate [P-124] Olanzapine-induced atypical neuroleptic malignant syndrome in an adolescent male with anorexia nervosa Hasret Ayyildiz Civan1, Senol Turan2, Didem Gulcu1, Cana Aksoy Poyraz2, Esra Pehlivanoglu3, Mehmet Kemal Arikan2, Fugen Cullu Cokugras1 1 Istanbul University Cerrahpasa Medicine Faculty Department of Paediatric Gastroenterology, Hepatology and Nutrition, Istanbul-Turkey 2 Istanbul University Cerrahpasa Medicine Faculty Department of Psychiatry 3 Istanbul University Cerrahpasa Medicine Faculty Department of Paediatrics Background: Anorexia nervosa (AN) is a serious mental illness of adolescents and young adults which involves fatal restriction of food intake and resulting physical, cognitive and social deteriorations. Antipsychotics have been especially used for weight gain and body focused delusional thoughts of these patients and neuroleptic malignant syndrome (NMS) is one of their rare fatal complications. We report a case of AN who developed NMS with a low dose of olanzapine. Case Presentation: A 17-year-old male referred by psychiatry department admitted with weight loss, restriction of food intake and intermittent binge eating followed by vomiting. On admission he had a BMI of 11.9 kg/m2. He achieved a weight gain of 10 kg with nutritional and cognitive behavioral therapy within the first four months. After a quarrel with his family, however, his vomiting recurred. In order to increase his weight and to prevent vomiting, olanzapine 5mg/day was initiated. On the second day of olanzapine therapy, he developed high fever (axillary 40ᴼC) unresponsive to antipyretics, muscle rigidity in extremities and stupor without any sign of meningeal irritation, infection and abnormal laboratory test results. Despite discontinuation of olanzapine, sporadic episodes of fever, muscle rigidity and elevated creatine kinase levels were observed even thirty days after the recovery, all of which ceased spontaneously within following five days. No other explanation could be made for his complaints at that time and he has been followed up in pediatric outpatient clinic. Conclusion: Although administration of olanzapine was discontinued, NMS persisted up to a month after the onset. Clinicians should bear in mind that the course of NMS may be heterogeneous. In addition, one should be careful while prescribing antipsychotic medications to paediatric cases and patients with AN who are severely underweight. Keywords: anorexia nervosa, neuroleptic malignant syndrome, olanzapine Conclusion: Although the outcomes of corrosive esophagitis with benzalkonium chloride may be fatal, a conservative approach may occasionally be sufficient owing to early diagnosis and neonatal wound healing with minor sequela. - 69 - [P-125] Intussusception in a newborn: a rare and unusual cause for intestinal obstruction Alper Aykanat1, Hatice Güllüelli1, Abdülhamit Tüten2, Ali Çay3, Osman Hacıosmanoğlu4 1 Department of Pediatrics, Zeynep Kamil Maternity and Children’s Training and Research Hospital, Uskudar, Istanbul, Turkey 2 Department of Pediatrics, Division of Neonatology, Zeynep Kamil Maternity and Children’s Training and Research Hospital, Uskudar, Istanbul, Turkey 3 Department of Pediatric Surgery, Avicenna Hospital, Esenler, Istanbul, Turkey 4 Department of Pediatrics, Avicenna Hospital, Esenler, Istanbul, Turkey Background&Aims: Intestinal obstruction is a life threatening emergency in neonatal period and is usually caused by necrotizing enterocolitis, meconium ileus and surgical causes. Intussusception is rarely reported in neonatal period as a cause of intestinal obstruction. We report a newborn presented with intussusception and wanted to underline the importance of accurate and timely diagnosis. Case: A 3350 g male baby was born to a G1P0 mother after 38 weeks of gestation. After routine newborn care, enteral feeding is started immediately. The newborn presented with bilious vomiting and progressive abdominal distension in first 8 hours of life and enteral feeding is terminated. Abdominal radiograph revealed dilated intestinal loops with air-fluid levels and raised suspicion to a possible intestinal obstruction. Abdominal ultrasound imaging diagnosed intussusception near ileo-cecal junction. The newborn was operated within postnatal 1st day. Intra-operative findings confirmed ileoileal intussusception, affected segment was resected and end-to-end anastomosis was made. The newborn was discharged on post-operative 10th day with full health. Conclusions: Intussusception in neonatal period is a rarely encountered entity and can easily be misdiagnosed unless a high degree suspicion is present. The etiology and pathophysiology of instussuseption remains unrevealed in newborns. Physical examination, laboratory tests and abdominal radiograph may not be sufficient for accurate diagnosis. Surgical procedures can be delayed due to late diagnosis. Ultrasound imaging is proved to be helpful and gains time in diagnosis. Keywords: Intestinal obstruction, Intussusception, Newborn [P-126] Isoniazid intoxication: Case report Hatice Öztürk, Arzu Aras, Aynur Bedel, Özlem Ketenci Altıkardeşler, Çağatay Nuhoğlu Haydarpaşa Numune Education and Research Hospital, Department of Paediatry Isoniazid (INH) is a bactericidal agent that is used for treatment and prophylaxis of tuberculosis infection. Exposure to INH in high doses may cause seizures and mental changes. A 16 years old male patient, was admitted to the pediatric emergency department with seizure. In his first examination, he was unconscious and had a generalized tonic-clonic seizure. After intravenous (IV) diazepam administration to the patient, seizure stopped, 15 minutes later unconsciousness relieved. In his medical and family history, there wasn’t no special finding except taking INH for the prophylaxis of tuberculosis for a week. When the family was questioned, we learnt that our patient was using ten pills once a day. We planned to give intravenous pyridoxine (B6) to the patient for the treatment. Since we had no intravenous pyridoxine form in this hospital, we gave oral pyridoxine (250 mg capsule). Following this treatment, in the second day of hospitalization, all of physical examination and laboratory findings relieved. All of the patients who are taken to the emergency departments of the hospitals with seizure or unconsciousness, should be evaluated for trauma exposure or medicine ingestion. They should be kept in mind for the etiology of seizures especially if the patients are not epileptic. Early diagnosis and treatment of INH intoxication has a great importance in order to decrease mortality and morbidity. Keywords: Isoniazid, seizure, pyridoxine [P-127] A Rare Infection In A Newborn: Meningococcal Meningitis Hatice Öztürk, Arzu Aras, Aynur Bedel, Umut Durak, Dilşad Koca, Çağatay Nuhoğlu Haydarpasa Numune Education and Research Hospital, İstanbul,Turkey Meningitis is inflammation of the arachnoid membrane and pia surrounding the brain and spinal cord. Neonatal period is a period which bacterial meningitis is more commonly seen than other ages. Neisseria meningitidis is an encapsulated gram-negative bacterium; there is no reservoir in other aliens in nature. It can cause either limited infection or septic shock syndromes. Although it is seen in all age groups, it is more common in children. After delivered from 22-year-old mother in expected date with normal spontaneous vaginal route, the male newborn was admitted to our clinic in 3730 g weight and on postnatal day 22 with fever. The patient with neonatal sepsis was hospitalized in intensive care unit for further examination and treatment. In history, there is no remarkable data except for a visit of patient’s relative returning from a pilgrimage. Peripheral blood smear supported sepsis. Following lumbar punction, cerebrospinal fluid(CSF) examination revealed abundant polymorphonuclear leucocytes. Antibiotic therapy was started. Gram-negative diplococcic was isolated in CSF culture. On the 7th day of treatment, control lumbar puncture was performed, 25 cells were counted. Control CSF cultures and blood cultures were evaluated. Visual and hearing tests were planned for possible complications. Invasive meningococcal disease can be seen in high risk population especially in younger than 2 years of children, 15-19 year-old adolescents, students and young adults in dormitories and military recruitments in army. Meningococcemia is still a systemic disease with high morbidity and mortality. Under 5 years of age, it - 70 - is responsible for 9.5% of all mortality in our country. By presenting this rarely seen infection in neonatal period, we pointed out the importance of diagnosis and treatment for meningococcal meningitis. Keywords: Meningitis, newborn, Neisseria meningitidis [P-128] Group A Streptococcal Meningitis in a Newborn: Transmission from Mother Hatice Sınav Ütkü1, Şifa Şahin1, Gamze Demirel2, Ayhan Taştekin2 1 Medipol University, Department of Pediatrics 2 Medipol University, Department of Neonatology Introduction: Group A Streptococci (GAS; Streptococcus Pyogenes) is an agent that may result in bacteremia, sepsis, menengitis and deep soft tissue infections and associated to 0,2-1 % of the meningitis cases. Here we report a newborn who hospitalized for meningitis and the etiological agent was GAS which was also detected at the throat culture of the mother. Case Report: A 22 days old term female neonate presented with fever and poor sucking for six hours. She was hospitalized for initial diagnosis of sepsis. In laboratory test results, infection markers increased and leukopenia was detected. We performed lumbar puncture which is full of leukocytes on microscopic examination. We started ampicillin and cefotaxime ampirically. Because of the deterioration of clinical condition, patient was intubated. We added inotropic agents related to developing hypotension, sodium replacement therapy was given for hyponatremia and phenobarbital administered for the convulsion. GAS reproduced in the cerebrospinal fluid and blood culture. We detected GAS in her mother’s throat culture. There are bilateral hypodens encephalomalacic areas especially in left cerebral hemisphere, also multipl hyperdens haemorrhagic areas detected in patients’ cranial computed tomography. EEGs report was bioelectric silence. She was discharged at 22th day of hospitalization. The patient is on follow up, she is now on 6th month of age, the neurologic examination is normal except microcephaly. Conclusion: GAS meningitis is a serious disease rarely seen in newborns. Parent and siblings of the patients may constitude the source of the infection. Clinicians should always consider GAS in the differential diagnosis of neonatal sepsis and meningitis and appropriate therapy should be given. Keywords: group a streptoccocci, newborn, meningitis [P-129] asymptomatic case: 16 month-old duchenne muscular distrophy Haticenur Kirar1, Ihsan Kafadar1, Cavid Serdarzade1, Attila Alp Gözübüyük1, Fatih Kirar2, Ozan Özkaya1 1 Department of Pediatrics, Okmeydanı Training & Research Hospital, Istanbul, Turkey. 2 Department of Neurosurgery, Haseki Training & Research Hospital, Istanbul, Turkey. Duchenne muscular dystrophy (DMD) is one of the most common neuromuscular disorders of childhood. Progressive degeneration of muscle is responsible for the clinic of patients. Symptoms begin at the age of 3–5 years. A case of a 16 month-old boy with DMD who was initially admitted the emergency department with febrile convulsion was reported. No symptoms and family history was noted. In his physical examination, bilateral stiffness of calf muscle(gastrocnemius) was interpreted as pseudohypertrophy. Other organ-systems were normal. Laboratory evaluation showed elevation of alanine amino transferase (ALT), aspartate amino transferase (AST), serum creatine kinase (CK) and lactate dehydrogenase (LDH). His chest X ray was normal. His genetic analysis revealed a deletion implying at 45-51 exons of the dystrophin gene. A molecular work-up of the parents showed his mother was the carrier. Genetic consultancy was given to the family. DMD is characterized by the progressive muscle weakness. It is caused by the mutation in dystrophin gene located on x chromosomes that’s affecting 1 in 3600 live male births. It is the main cause of the progressive muscle weakness occurring clinical of the patients. Symptoms include trouble in walking, loss of reflexes, difficulty in standing up, poor posture, bone thinning, scoliosis, mild mental impairment, breathing difficulties, swallowing problems, lung and heart weakness. In our patient there are no specific symptoms of DMD. He presented only elevation of amino transferase (ALT and AST) which are markers of hepatocellular injury but are highly concentrated in muscle cells. We aim to report this case, asneuromuscular disorders may be suspected in the patient who identified only with high amino transferase. Keywords: duchenne muscular distrophy, aminotransferase, asymptomatic [P-130] Effect of Cultural Differences of Nursing Care Hazal Hazal Huzmeli1, Ayşe Ferda Ocakci2 1 Amerikan Hospital 2 Koç University School of Nursing Summary: Earth; 21th century with the name “Big Village”. In this age we live in a different nation every moment, we can encounter people from different race or ethnicity. One of the best examples are in the hospital. Historical and cultural journeys, especially in “health tourism” because of the spread of different cultures in our confrontation is inevitable. Turkey said “We can not ignore the role of health tourism. The historical building, both with people we meet with many ethnic origins in domestic and abroad because of advanced hospitals. As - 71 - well as the last century wars with globalization in almost all regions of the world, ethnic conflicts, environmental crises, repressive regimes, economic collapse situations like many of the people in the country or as refugees migrants to other countries, is caused to migrate voluntarily or compulsory. In this case the world in different cultures and subcultures of individuals are emerging multicultural population structure composed of families and groups. Nursing disease is not concerned with the effects on the patient’s disease it is the most affected by health disciplines to cultural differences. It will adversely affect the rights of patients ignore their culture because they are all with their own culture. Objectives: Nursing Care of “cultural differences” in creating awareness. Objective: Use in nursing care plans of cultural differences and to improve the quality of care. Methods: Review Article Keywords: cultural differences, nursing care, nursing effects of cultural diversity [P-131] A rare benign condition that should be remembered in differential diagnosis of hyperamylasemia in children: Macroamylasemia Melike Emiroğlu1, Halil Haldun Emiroğlu2, Hikmet Akbulut3 1 Department of Pediatric Infectious Disease,Faculty of Medicine,Selcuk University, Konya, Turkey 2 Department of Pediatric Gastroenterology, Hepatology and Nutrition,Faculty of Medicine,Selcuk University, Konya, Turkey 3 Department of Pediatric, Faculty of Medicine,Selcuk University, Konya, Turkey Aim: Macroamylasemia is a benign hyperamylasemic condition caused by circulating macroamylase complexes of pancreatic or salivary amylase bound to immunoglobulins (IgG, IgA), which can be filtered very slowly from the blood by the renal glomeruli. Although macroamylasemia accounts for 2.5% of hyperamylasemic conditions and has been associated with a variety of diseases including celiac disease, autoimmune disorders, rheumatoid arthritis, ulcerative colitis and lymphoma. Also, 1% of healthy subjects have macroamylasemia and it requires no treatment, may be transient. In macroamylasemia, the amylase/creatinine clearance ratio described below is usually <1% (normal 1-5%), and the urine amylase is usually low. Because of it is a rare condition in children with hyperamylasemia, we report a healthy boy with macroamylasemia. Case: A 1.5 year old boy was admitted to the hospital for nausea and vomiting associated with hyperamylasemia and suspected acute pancreatitis. His physical examination and blood biochemistry analysis were normal except high level of serum amylase. Ultrasonographic examinations for pancreatitis and/or salivary glands diseases (parotitis, etc.) were normal. Screening for viral infections including mumps IgM was also normal. There was no member with high level of serum amylase in his family. We confirmed elevated serum amylase, normal serum lipase, and very low amylase clearance/creatinine clearance ratio (0.3%), consistent with hyperamylasemia. Conclusion: If macroamylasemia is kept in mind as an etiology of amylase elevation in the children without elevation of serum lipase level, unnecessary further diagnostic tests will be precluded. Keywords: macroamylasemia, pediatric [P-132] Is MUAC cut-off level must be specific for each country in diagnosis of malnutrition? Sedat Özdemir1, Melike Emiroğlu2, Fatih Kara3, Mehmet Sinan İyisoy4, Hikmet Akbulut1, Seral Navdar1, Halil Haldun Emiroğlu5 1 Department of Pediatric, Faculty of Medicine,Selcuk University, Konya, Turkey 2 Department of Pediatric Infectious Disease,Faculty of Medicine,Selcuk University, Konya, Turkey 3 Department of Publıc Health, Faculty of Medicine,Selcuk University, Konya, Turkey 4 Head of Medical Education and Informatics Department,Meram Faculty of Medicine,Necmettin Erbakan University, Konya, Turkey 5 Department of Pediatric Gastroenterology, Hepatology and Nutrition,Faculty of Medicine,Selcuk University, Konya, Turkey Objective: We aimed to assess the usability of midupper arm circumference (MUAC) measurement in malnutrition diagnosis of children aged between 1 and 5 years-old and, its relationship with other classifications. Material-Method: 1500 children between 1-5 yearsold, who admitted to the Selcuk University Faculty of Medicine, polyclinics of the Department of Pediatrics, during June 01, 2014/September 30, 2014 period, were included by simple random sampling method. Anthropometric and MUAC measurements recorded and malnutrition levels have been determined, according to the Gomez, Waterlow and WHO classifications. MUAC cut-off values in respect of malnutrition were calculated for Gomez, Waterlow and WHO classifications by the means of ROC analysis. The sensitivity, specificity, PPVs, and NPVs of the MUAC were determined according to either malnutrition classifications. Results: MUAC had better compatibility with Gomez classification in respect of sensitivity and specificity. In the diagnosis of malnutrition, cut-off point of MUAC for Gomez and Waterlow classifications is determined to be 14.95 cm; for WHO weight for age z score to be 13.95 cm; for WHO height for age z score to be 14.55 cm; and for all classifications to be 15.15 cm. When we compared the compatibility of MUAC with other malnutrition classifications, cut-off point value as 11.5 cm produced better results than 11.0 cm in respect of sensitivity, specificity, PPVs, and NPVs in the diagnosis of severe malnutrition. Conclusion: For the diagnosis of severe malnutrition, 11.0 cm value is used in some countries as a MUAC cutoff point, but we think that preferring 11.5 cm value - 72 - is more suitable for our country. Although 12.5 cm is routinely used as MUAC cut-off value for the diagnosis of malnutrition, 15.15 cm cut-off value may increase the sensitivity of this method. Keywords: Malnutrition, mid-upper arm circumference(MUAC), malnutrition classifications [P-133] Is there a relationship between nutritional characteristics and malnutrition among the age group of 1–5 years old children? Seral Navdar1, Melike Emiroğlu2, Fatih Kara3, Gülay Önal3, Hikmet Akbulut1, Halil Haldun Emiroğlu4 1 Department of Pediatric, Faculty of Medicine,Selcuk University, Konya, Turkey 2 Department of Pediatric Infectious Disease,Faculty of Medicine,Selcuk University, Konya, Turkey 3 Department of Publıc Health, Faculty of Medicine,Selcuk University, Konya, Turkey 4 Department of Pediatric Gastroenterology, Hepatology and Nutrition,Faculty of Medicine,Selcuk University, Konya, Turkey Objective: Malnutrition is an important health problem. The aim of this study was to investigate the relationship between nutritional characteristics and malnutrition among the age group of 1-5 years old children in Konya, a city in the Central Anatolia of Turkey. Material-Methods: A total of 435 children between 1-5 year-old who admitted to Pediatrics Outpatients Clinic of Selcuk University Faculty of Medicine between January 2013 and 2015 were included in the study. The children divided into two groups according to Waterlow or Gomez Classifications: (1) malnutrition group (215 children); (2) control group (220 children). Both groups were evaluated with 51 item questionnaire that include sociodemografic and socioeconomic features of families, nutritional characteristics and eating behaviors of children, nutrition knowledge and child feeding style of mothers. Results: The main cause of malnutrition is the error in the practice during additional foods transition period subsequent to breastfeeding. During the transition period to supplementary feeding, there were more mistakes in the patients with malnutrition than the control group. Eating behavior abnormalities and inappropriate feeding styles of mothers were apparently high in malnutrition group. Conclusion: The training of mothers about supplementary feeding may reduce the incidence of malnutrition. Keywords: Breastfeeding, malnutrition prevalence, nutrition education [P-134] Cyst hydatid case presenting with thorax deformity Yaşar Şen1, Hikmet Akbulut2, Emine Ayça Cimbek1, Yavuz Köksal3, Orhan Özbek4 1 Department of Pediatric Endocrinology,Faculty of Medicine,Selcuk University, Konya, Turkey 2 Department of Pediatric, Faculty of Medicine,Selcuk University, Konya, Turkey 3 Department of Pediatric Oncology, Faculty of Medicine,Selcuk University, Konya, Turkey 4 Department of Radiology, Meram Medical Faculty, Necmettin Erbakan University, Konya, Turkey Introduction: Hydatid cyst, endemic in many regions, is a parasitic disease caused by echinococcosis granulosus.According to the affected organ, different clinical symptoms are evident. It has a prevalence of 50-400/100 000 and an incidence of 3.4/100 000 in our country. Diagnosis is based on the cyst determined during radiological examination.In the present case, a 6 year old girl referring to our clinic due to thorax deformity with distinct hepatomegaly determined during physical examination and diagnosed with hydatid cyst is presented. Case: A 6 year old girl referred to our outpatient clinic due to thorax deformity which has been recognised for 4 years. On physical examination, her height and weight percentiles were normal. In the abdominal region, 5-6 cm below the ribs, a palpable mass with well circumscribed borders adjacent to the liver was determined. Moreover, the patient had also a chest wall deformity. Other systems were considered to be normal. Abdominal ultrasonography (USG) and magnetic resonance imaging (MRI) of the patient revealed a 151X113 mm cystic structure, membranous lesion (hydatid cyst), pushing the kidney inferior without well circumscribed liver parenchyma border with tiny (mm) moving echogenities (daughter vesicles) elongating papillary. Complete blood count, biochemical markers were normal. Accompanied with USG and fluoroscopy, puncture-aspiration-injectionre-aspiration (PAIR) was applied to the patient. Discussion: In the present case, the detailed physical examination of the patient referred to our clinic due to chest wall deformity revealed hepatomegaly due to cyst hydatid as the main diagnosis. We think that severe hepatomegaly and abdominal distansion led to patient’s thorax deformity present since early childhood to be more prominent and thus to the referral to our clinic. Liver hydatid cyst diagnosis was made based on the careful examination and diagnostic tests made. Hereby, the importance of detailed patient history and physical examination is to be highlighted once again. Keywords: Thorax deformity, hydatid cyst, children - 73 - [P-135] Mosaic 47 XYY syndrome presenting with tall stature and macrogynecomastia Emine Ayça Cimbek1, Sevil Arı Yuca1, Yaşar Şen1, Nadir Koçak4, Hikmet Akbulut2, Fuat Buğrul1, İlhan Çiftçi3, İlter Paydur2 1 Department of Pediatric Endocrinology,Faculty of Medicine,Selcuk University, Konya, Turkey 2 Department of Pediatric, Faculty of Medicine,Selcuk University, Konya, Turkey 3 Department of Pediatric Surgery, Faculty of Medicine,Selcuk University, Konya, Turkey 4 Department of Medical Genetics, Faculty of Medicine,Selcuk University, Konya, Turkey 47,XYY syndrome is a chromosomal abnormality associated with an increased risk of learning disabilities, delayed development of speech, language and motor skills, tremors or motor tics, behavioral and emotional difficulties. These characteristics vary widely among affected individuals. Boys with this condition may be taller than average, have severe acne during adolescence but gynecomastia is not a very common finding. Here we present a boy diagnosed as mosaic 47 XYY syndrome associated with tall stature and macrogynecomastia. A 13-year-old male patient visited our hospital for assessment of his tall stature and breast development. He was born with a birth weight of 4,100 g. His family history revealed members with very tall stature. His height and weight were both above the 97th percentile. On physical examination, he was found to have severe acne on his face, a Tanner stage of 4 and bilateral macrogynecomastia. His routine biochemistry, thyroid function tests, insulinlike growth factor Ievel and other pituitary hormone levels were all within normal ranges. His bone age was consistent with his chronological age, and his predicted adult height was approximately 200 cm. Sella magnetic resonance imaging showed a microadenoma 8x6 mm in diameter. He was diagnosed with XYY syndrome with a chromosome test result showing a 47,XYY (%93)/46XY (%7) mosaism and referred to the pediatric surgery department for the assessment of macrogynecomastia. When evaluating boys with learning disabilities and behavioral problems a chromosome study should be carried out if they have additional physical abnormalities such as tall stature (although it may be familial) and gynecomastia. In that case XYY syndrome should be ruled out keeping in mind that they are very responsive to early intervention and supportive treatment. Keywords: children, 47XYY, gynecomastia [P-136] A case report: Miller Fisher variant of Guillan Barre Syndrome and anti GQ1b antibody positivity Hilal Fevziye Durmaz Şimşek, Tamay Gürbüz, Çiğdem Sağ, Umut Durak, Narin Akıcı, Çağatay Nuhoğlu depertment of pediatrics, Haydarpasa Numune Hospital, Istanbul, Turkey Guillan- Barre Syndrome is an acute post-infectious demyelinating polyneuropathy with an acute onset characterised by genereally fast progressive muscle weakness and paraesthesia. GBS is diagnosed by clinical, laboratory and neurophysiological findings.(1) Gbs has different clinical forms characterised by the different involvement of motor and sensory axons of peripheral nerves and the autonomic nervous system. (2) Miller Fisher syndrome is a subtype of GBS which characterized by external ophthalmoplegia, ataxia and muscle weakness with areflexia. Incomplete forms include acute opthalmoplegia without ataxia and acute ataxic neuropathy without ophthalmoplegia. Cerebrospinal fluid findings and electrophysiologic features are similar to those in protype of GBS. Anti-gangliosid anti bodies, mostly ıg g type, in patient serum support the diagnosis.Ig G anti-GQ! B antibody is one of the best studied antibodies whichare specifically associated with variant of Fisher Syndrome. AntiGQ1b monoclonal antibody specifically immunostains paranodal myelin of human cranial nerves innervating extraocular muscles and some large neurons in dorsal root ganglia. (3) Thus, the anti GQ1b antibodies may cause ophthalmoplegia and ataxia by binding to the regions where GQ1b is densely localized. Keywords: guiilan barre syndrome, miller fisher syndrome, anti gq1b antibody [P-137] Bone marrow suppression in an adolescent with toxoplasmosis Huray Kok, Hatice Eren, Dilsad Koca, Zehra Esra Onal, Cagatay Nuhoglu Haydarpasa Numune Education and Research Hospital Introduction: Toxoplasma gondii is an intra cellular protozoan parasite. Infection in humans occurs through the ingestion of raw or low cooked meal that contains cysts, or congenitally tranplacentally from an infected mother during pregnancy. Case: Seventeen years old female was admitted to our policlinics with nausea and vomiting. In her history, she had been using corticositeroid, pyrimethamine and thyrimthmine-sulfamethoxazol for the diagnosis of chorioretinitis due to toxoplasmosis. Her physical examination revealed jaundice of skin, she had no splenomegaly or hepatomegaly and lymphadenopathy. Neurologic examination was normal. Laboratory findings showed anemia and thrombocytopenia with Hb:5,9 gr/dl, Plt: 109.000/ml. Peripheral blood smear showed hypochromic erythrocytes with hypersegmented neutrophils, there was no atypical cell. Reticulocyte, hemoglobin electrophoresis, B12 - 74 - levels, PT, ANA (Anti-nuclear anticore), RF (Romatoid factor), EBV IgM-IgG (Epstein Barr virus) levels were within normal limits. Serum folic acid level was 1,5 ng/ ml, lower than normal. Oral folic acid replacement was used. Bone marrow aspiration evaluation was evoked bone marrow due to steroid treatment. It was considered as secondary bicytopenia caused by toxoplasmosis. Following up the patient, since anemia and thrombocytopenia got worse, the patient was transfused with erythrocyte and thrombocyte suspensions. Corticosteroid treatment stopped. Her second bone marrow aspiration was within normal limits. Discussion: The etiology of pancytopenia or bicytopenia has a wide distribution in childhood. In children viral infections are the main causes of bone marrow suppression, but in can be the result of malign disorders as well. It can also be due to drugs, chemotherapy and radiotherapy. Pancytopenia or bictopenia are the clinical manifestations of toxoplasmosis. Bone marrow suppression is the main complication of treatment with pyrimethamine. But we demonstrated serological tests and bone marrow aspiration for differential diagnosis of etiology. Keywords: toxoplasmosis, thrombocytopenia, pyrimethamine [P-138] Pericardial effusion in diarrhea positive atypical hemolytic uremic syndrome Hüseyin Bardak1, Aylin Gençler2, Merve Korkut1, Havva Evrengül2, Dolunay Gürses3, Selçuk Yüksel2 1 Department of Paediatrics, Pamukkale University School of Medicine Hospital at Denizli, Turkiye 2 Department of Paediatrics, Division of Paediatric Nephrology, Pamukkale University School of Medicine Hospital at Denizli, Turkiye 3 Department of Paediatrics, Division of Paediatic Cardiology, Pamukkale University School of Medicine Hospital at Denizli, Turkiye Introduction: Besides several extra-renal manifestations in diarrhea positive (D+) hemolytic uremic syndrome (HUS), cardiac involvement is a rare but significant complication. We present a severe pericardial effusion in a child with D+ atipical HUS. Case: A previously healthy thirteen years old girl presented with four day history of bloody diarrhea. At presentation, she was unwell, blood pressure was 120/80 mmHg, has no edema. The initial blood tests revealed white blood cells (WBC) 12610/mm3, haemoglobin 11,9 gr/dL, platelet 63000/mm3, urea 49 mg/dL, creatinine 1,41 mg/dL. Urinalysis showed proteinuria and microscobic heamaturia. Abdominal ultrasound revealed both kidneys with normal size and echogenity, widespread free fluid and diffuse mucosal edema on bowels. Within 24 hours, she developed anuria. Control lab results showed anemia, trombocytopenia and haemolysis (Heamoglobin 7gr/dL, LDH 2600 IU/L, haptoglobuine <10 mg/dL). Blood smear showed fragmanted erythrocytes. She was determined as D+ HUS. O157:H7 returned negative, ADAMTS-13 activity was 15% (40-130). Within four days, she needed haemodialysis due to rapid urea and creatinine rise and diuretic resistant anuria. She was instituted Eculizumab therapy due to ongoing anuria and worsening renal functions despite haemodialysis. Urine output was observed on second day of Eculizumab infusion. She encountered hypotension and troponine-T rise (0,016 ng/mL) on day 28. Echocardiography revealed pericardial effusion without tamponate and left ventricle ejection fraction 70%. She had undergone a pericardiosyntesis right after we had seen progression on effusion with control echocardiograpies. 280 ml of pericardial fluid was drained. She was then observed with antibiotics and anti-inflmatory treatment. Pericardial effusion was diminished on day 32 of Eculizumab treatment and she was discharged with plans of Eculizumab protocol. Conclusion: Cardiac manifestations in D+ HUS cases are rare. These should be considered and promptly evaluated with echocardiograpyh when patients encounter abrupt chest pain while renal functions are recovering. Keywords: pericardial, effusion, hus [P-139] A Rare Type Of Situs Inversus: Isolated Levocardia Ibrahim Mert Erbaş, Ozge Demirel, Helen Bornaun, Rengin Şiraneci, Nail Uzunlulu, Özlem Öner Kanuni Sultan Süleyman Eğitim ve Araştırma Hastanesi, İstanbul, Turkey Background&Aims: Situs inversus is a congenital condition in which the major visceral organs (within the thorax and abdomen) are either reversed or mirrored from their normal positions. When the heart remains on the normal left side of the thorax in the presence of abdominal situs inversus, the condition is known as situs inversus with isolated levocardia. The estimated reported incidence is 1 per 22,000 in the general population and from 0.4% to 1.2% in all patients with congenital heart disease. Severe forms of congenital heart defects are almost always associated with this condition. Splenic defects such as asplenia and polysplenia are examples of this anomaly. Polysplenia is characterized by a tendency for bilateral left-sidedness, including bilateral two-lobed lungs, interruption of the inferior vena cava (IVC) with azygos continuation, and complex cardiac anomalies. The only information regarding the outcome of patients with this anomaly has been that of case reports on adults and children in whom the diagnosis was made incidentally or after abdominal surgery, particularly for a volvulus or bowel obstruction. Moreover, according to published data, the outcome is exiguous in children and adults. In only approximately 5% to 13% do patients survive for more than 5 years, mainly because of the severity of an associate cardiac abnormality. Keywords: Situs, Isolated, Levocardia - 75 - [P-140] A Cause Of Treatment Resistant Bronchopneumonia: H Type Tracheooesophageal Fistula Irem Ceren Arslan, Gözde Akın, Emel Karaoğlan, Hüseyin Aldemir, Ibrahim Mert Erbaş Kanuni Sultan Süleyman Eğitim ve Araştırma Hastanesi, İstanbul, Turkey Background&Aims: Oesophageal atresia and tracheo‐oesophageal fistula (OA/TOF) are common life‐threatening malformations with an incidence of approximately 1 in 3500 births. The etiology of OA/TOF is unknown in the majority of cases. In approximately half of the cases (syndromic oesophageal atresia), there are other associated anomalies, with cardiac malformations being the most common.1 It is hard to diagnose H type fistula in neonatal period, which is a subtype of TOF without oesophageal atresia.2 These patients can live until adult ages, without any diagnose. Most of them have recurrent respiratory system infections, cyanosis and apirations during feeding.3 In our case, we reported a 45-days-old male patient that had treatment resistant bronchopneumonia, and the further studies showed us H type tracheo‐oesophageal fistula. Keywords: Tracheo-oesophageal fistula, h type, bronchopneumonia [P-141] Rasmussen encephalitis: hard diagnosis, harder therapy Judit Szamosújvári1, Beáta Rosdy1, Katalin Kollár1, Judit Móser1, Mónika Mellár1, Éva Kovács2, Gabriella Kiss3, Gábor Rudas4, György Várallyay4 1 Neurology Department of Heim Pál Children Hospital, Budapest, Hungary 2 Department of Radiology, Heim Pál Children Hospital, Budapest, Hungary 3 ICU, Heim Pál Children Hospital, Budapest, Hungary 4 Semmelweis Medical School MR Research Institute Introduction: Rasmussen encephalitis (RE) is an extremely rare autoimmune chronic inflammatory neurodegenerative disease affecting a single cerebral hemisphere, causing progressive neurological deterioration and intractable seizures. Antiepileptic and immunmodulatory treatment can be tried, but surgery (hemisphaerotomy), if applicable is the treatment of choice. Case: 9-year-old boy presented to our department with fever provoked repetitive grand mal seizures in sleep. Later in the disease course, his seizures progressed to epilepsia partialis continua (EPC) with right sided hemiparesis and speech difficulties. EPC did not respond to antiepileptic treatment. MRI of the brain showed on T2/FLAIR hyperintensive signals in cortical and subcortical region of the left temporal lobe, suggesting the diagnosis of RE. Later cortical atrophy developed in this region. PET-CT presented focal hypometabolic activity in that region, as well. Because eloquent cortex was affected, surgery could not be a solution. We started immunmodulatory therapy using steroid pulse therapy with plasmapheresis. Because of septic complications we switched to intravenous immunoglobulin (1,2 g/kg/dose) monthly, combined with long-term oral steroids. Seizure freedom was achieved and hemiparesis disappeared. The patient is on these medications even up to date. We taper steroids. Conclusion: Immunmodulatory treatment combined with antiepileptic drugs should be considered in therapy of RE. Presurgical evaluation must be performed. If surgery is applicable, the timing of it is a critical point in the treatment decision. Post-surgical neurological deficits are frequently equivalent to those inevitably resulting from RE. Keywords: Rasmussen encephalitis, diagnosis, therapy [P-142] A Life-Threatening Retropharyngeal Abscess Adem Binnetoğlu1, Yavuz Gündoğdu1, Kıymet Keçelioğlu Binnetoğlu2, Ali Cemal Yumuşakhuylu1, Tekin Bağlam1, Murat Sarı1 1 Marmara University Pendik Treaning and Research Hospital, Department of Otorhinolaryngology Head and Neck Surgery, Istanbul-TURKEY 2 Marmara University Pendik Treaning and Research Hospital, Department of Pediatrics, Istanbul-TURKEY The incidence of Retropharyngeal abscess (RPA) is increased from 0.1 cases per 10,000 to 0.22 cases per 10,000 in 2000 to 2009 [1] The reasons are thought to be due to bacterial resistance as well as improving investigative modalities (some of RPAs were misdiagnosed and treated with the antibiotics) [1]. Children with RPA are usually caused by abscess formation within inflamed local lymph nodes during or after the upper respiratory tract infections[2]. RPAs are potentially life-threatening conditions in both paediatric patients and adults. They can result in local, regional and systemic complications, as follows airway obstruction, mediastinitis, jugular vein thrombophlebitis, cranial nerve dysfunction, cervical osteomyelitis, meningitis, sepsis, disseminated intravascular coagulopathy (DIC), and death [3,4]. These complications have become a less likely due to appropriate antibiotic treatment [3,4]. In our case, the patient have cervical lymphadenopathy and swelling, odynophagia, stertor and difficulty in neck movement. His breathing sound gets more noisy and WBC count and CRP levels were not decreased even under intravenous (IV) antibiotic treatment. Radiological evaluation revealed RPA with 60x30 mm in diameter. We performed transoral abscess drainage. Post-operative follow-up showed dramatical clinical improvement. RPA is an increasingly common infection that leads to admissions to pediatric and emergency departments. RPA is a potentially serious illness that should allert physicians. They have to consider RPA in the differencial diagnosis with other diseases that show the same clinical manifestations. Topic: Retropharyngeal abscess Keywords: Life-threatening conditions, Antibiotheraphy, Transoral Drainage - 76 - [P-144] Keywords: Life-threatening conditions, Antibiotheraphy, Transoral Drainage Comparism between transcutaneous and serum bilirubin levels of neonates at Delivery Room [P-143] A Rare Disease: Subtelomeric Deletion of Long arm of 1st Chromosome in 4 Year Old Patient Koray Hacıoğlu1, Helen Bornaun2, Tuğçe Kalaycı Oral1, Zeynep Ocak3, Kazım Özatrhan2, Fatma Ekici3 1 Department of Pediatrics, Kanuni Sultan Süleyman Reseach and Training Hospital,İstanbul,Turkey 2 Department of Pediatric Cardiology, Kanuni Sultan Süleyman Reseach and Training Hospital,İstanbul,Turkey 3 Department of Medical Genetic and Birth Defects, Kanuni Sultan Süleyman Reseach and Training Hospital,İstanbul,Turkey Background&Aims: Methods: Subtelomeric FISH analysis Results: Subtelomeric deletion was detected in long arm of 1st chromosome Conclusions: Distal subtelomeric region of long arm of 1st chromosome is associated with characteristic facial appearance and accompanying malformations. Characteristic findings are: round facies, short neck, turned-down the corners of the mouth, epicanthic fold, long upper lip and a short philtrum, low set ears, micrognathia, microcephaly, abnormal hand and feet structure, various cardiac and genital anomalies, mild or severe mental-motor retardation, hypotonia, seizures, epilepsy, skeletal anomalies, psychomotor retardation and growth retardation. Deletions in distal subtelomeric region of long arm of the 1st chromosome are very rarely seen and firstly reported by Mankinen and friends. When clinical findings of our 3 years and 6 months old patient, who was under examination due to hypotonia and cardiac murmur, are evaluated, and pathology in all cardiac valves found at echocardiography, karyotype analysis is planned with suspicion of possible genetic disease. Patient is found to have normal karyotype analysis, performed for the differential diagnosis of genetic diseases. Subtelomeric FISH analysis has been performed when detailed dysmorphic examination revealed possible subtelomeric region changes. Subtelomeric deletion was detected in long arm of 1st chromosome. With his case, we would like to emphasize the importance of adding subtelomeric region investigations to karyotype analysis in hypotonic infants, since the latter may be insufficient. Keywords: FISH analysis, hypotonia, subtelomeric deletion Mürşide Uysal, Yelda Türkmenoğlu, Kyaw Zin Latt, Ensar Duras, Doygoameneh Ghare Mashggharavi, Ozan Özkaya SB Okmeydanı Eğitim ve Araştırma Hastanesi,Çocuk Sağlığı ve Hastalıkları Kliniği, Istanbul Background&Aims: More than 60% of newborns develop clinical jaundice in the first week of life and pathologic levels of bilirubin may lead to bilirubin encephalopathy. Because of its non-invasiv procedure, trancutaneous bilirubin measurement becomes more preferrable than serum bilirubin, but there are discussions about reliability. The aim of this study is to evaluate the compactibility between transcutaneous and serum bilirubin levels of neonates at delivery room of our hospital. Methods: The study was done at Pediatrics Department of Okmeydanı Research and Training Hospital. Transcutaneous bilirubin levels were measured from forehead and sternum of neonates of ages between 0-7 days, healthy, late preterm and term(above 35GW) by Bili Check metre. The results were compared with venous bilirubin levels. The same procedure was performed to neonates who lost weight more than 5% of their birth weight. Results: Out of 38 neonates, 20 were male(52,6%) and the birth weights vary from 2265 to 4270 grams (median 3252.11±505.75 gram).Serum bilirubin levels range between 6,7- 15,1 mg/dl. The study found out statistically and significantly positive corelation between serum bilirubin and transcutaneous sternum bilirubin levels 78,4%, between serum bilirubin and transcutaneous forehead bilirubin levels 73,4%, and between transcutaneous sternum bilirubin and transcutaneous forehead bilirubin levels 75,7% (p:0.001; p<0.01). There was no statistics and significant correlarions between serum bilirubin and transcutaneous bilirubin levels (sternum and forehead) bilirubin levels of the neonates who lost weight more and less than 5% of their birth weight (p>0.05).Three of neonates who had high bilirubin levels according to bilirubin nanograms were carried out phototherapy. Conclusions: Transcutaneous bilirubin measurement is a cheap, non invasive and safe method to detect the bilirubin levels of neonates before disharge from the hospital. But in the cases with significant hyperbilirubinemia, serum bilirubin level is more reliable for treatment protocols. Keywords: neonate, transcutaneous bilirubin - 77 - [P-145] A rare causes of cholestasis: Arthrogryposisrenal tubular dysfunction-cholestasis syndrome Yelda Türkmenoğlu1, Yeşim Acar1, Fatiih Cemal Özdemir1, Kyaw Zin Latte1, Ralfi Singer2, Erdal Adal1 1 Department of Pediatrics,Ministry of Health Okmeydanı Research and Training Hospital, Istanbul 2 Department of Dermatology,Ministry of Health Okmeydanı Research and Training Hospital, Istanbul Arthrogryposis- renal tubular dysfunction and cholestasis (ARC ) syndrome, the association of arthrogryposis, renal tubular dysfunction and cholestasis is a rare autosomal recessive multisystem disorder. Additional features are severe failure to thrive, ichthyosis, abnormal platelets, diarrhoea, recurrent infections, mild dysmorphic signs, nephrogenic diabetes insipidus, hypotonia, dysgenesis of corpus callosum and nerve deafness.Gissen et al. identified a mutation in VPS33B gene on chromosome 15q26.1. Most of patients die because of pneumonia, septicemia, acidosis, bleeding or dehydratation in the first year. Here we report a case of ARC syndrome from Turkey diagnosed by genetic analysis instead of invasive biopsies, but she died three years old age because of septicemia. Keywords: cholestasis, ichthyosis, renal tubular dysfunction [P-146] Mediastinal tumor; as case report Mahir Tıras, Ozlem Bostan Gayret, Ozgül Yiğit, Meltem Erol, Leyla Besel Bagcilar Treaning and Research Hospital, Pediatric Department, Istanbul, Turkey Background&Aims: Mediastinal masses in children constitute a heterogeneous group of malignant and benign neoplasms. Neurogenic tumors are reported to be the second most common pediatric mediastinal malignancy seen in 21%- 44% of cases. Neuroblastoma is most common and occurs in children two years of age or younger. In this report we present a case who applied with abdominal pain and diagnosed neuroblastoma with biopsy. Case: A five years old girl, whose personal and family medical history revealed no significant findings, admitted to pediatric emergency clinic with abdominal pain. Her heart rate, blood pressure and body temparature were normal. In his physical examination, breathing sounds were not heart in right hemithoraces. İn her chest radiography, opacity was seen completely in right lung. Her thorax tomography was compatible with mediastinal mass. According to her mediastinal biopsy result, she was diagnosed neuroblastoma. In her laboratory findings; Hb:10.57 gr/dl, WBC: 22000/mm3, PLT: 317000/mm3, LDH: 2786 U/L, uric acid: 2.6 mg/dl, CRP: 123 mg/L. neuron-specific enolase level was 309 ngr/ml. Other blood analysis were normal. She referred pediatric oncology clinic. neuroblastoma in differential diagnosis of pediatric mediastinal mass. Keywords: Mediastinal masses, neuroblastoma, childhood [P-147] Presentation of Atypical Hemolytic Uremic Syndrome Maltam Baghirova, Zehra Esra Önal, Tamay Gürbüz, Mustafa Behçet Şimşek, Çağatay Nuhoğlu Department of Pediatrics, Haydarpasa Numune EAH, Istanbul, Turkey Background&Aims: Microangiopatic hemolytic anemia, thrombocytopenia, fever, central nervous system abnormalities and acute renal failure are five main components of the Hemolytic Uremic Syndrome (HUS). Atypical HUS is related to genetic mutations and may occur without a gastrointestinal prodrome. Atypical HUS generally leads to poor outcomes and may advance to end-stage renal disease or permanent brain damage in approximately 50% of the patients. Methods: A four-year-old girl was admitted to our hospital with complaints of abdominal pain and high grade fever. She was given antibiotic medication. Edema and petechial rashes occurred on her skin over time. Due to these symptoms, we first evaluated the disease as meningococcemia. However, her blood count showing values as “HGB: 12.1, PLT: 70.000/uL, APTT: 19.4, PT: 1.61 and INR: 1.68”, the appearance of blood in the urine (macroscopic hematuria) and absence of diarrhea led us to give presumptive diagnosis as atypical HUS. A further more detailed blood test resulted in following Findings: microangiopatic hemolytic anemia with a negative Coombs’ test and thrombocytopenia (<40000/ uL). Afterwards, plasmapheresis, eculizumab, freshfrozen plasma was administered at a dose of 10 ml per kilogram on day in intensive care unit. Results: She had normal level of blood pressure and was fully conscious. Despite the therapy, her edema and thrombocytopenia did not resolve. Conclusions: This case underlines the limited understanding of clinical features of atypical HUS. Since it is a rarely seen disease, the diagnosis is difficult. Even if the right diagnosis is given, genetic testing is of great importance in deciding treatment options, but these tests are not widely available. This case highlights the need for high level of suspicion and more in-depth research towards better treatment alternatives keeping in mind the mortality it may cause. Keywords: Hemolytic Uremic Syndrome, atypical HUS Conclusions: This case presented to emphasize the - 78 - [P-148] A case of empyema necessitatis in a child with M. tuberculosis Manolya Acar1, Murat Sutcu1, Ezgi Topyildiz1, Tansu Salman2, Hacer Akturk1, Gonca Erkose Genc3, Bulent Oguz Erol4, Nuran Salman1, Ayper Somer1 1 Istanbul University Istanbul Medical Faculty, Pediatric Infectious Diseases 2 Istanbul University Istanbul Medical Faculty, Pediatric Surgery 3 Istanbul University Istanbul Medical Faculty, Microbiology and Clinical Microbiology 4 Istanbul University Istanbul Medical Faculty, Radiology Introduction: Empyema may extravasate outside the thorax cavity under pressure. This is known as empyema necessitatis (EN). It rarely occurs in children. Herein we report a child with EN due to pulmonary tuberculosis (TB). Case report: An 11-year-old boy presented with recently occurrence of swelling on his chest wall. He had been hospitalized for plevral empyema a year ago and his father had been diagnosed with cavitary TB at that time. Tube thoracentesis had been performed, and acid resistant stain for mycobacterium reported as negative from exudative material. Interferon gamma releasing test (IP-10) and tuberculin skin test had been positive. Anti tuberculous treatment (isoniazide, rifampisin, pyrazinamide, ethambutol) was initiated and he was discharged with recovery. After that he did not come to regular follow-up. Retrospectively we found, he had refused to take his medication and plevral fluid culture had come positive for M. tuberculosis. On admission, systemic examination was unremarkable other than a nodular area on his chest. X- ray showed small infiltration in left lung parenchyma without effusion. Thorax ultrasound and CT revealed 5cm diameter of intense material collection on thorax wall communication with pleural cavity. Acid resistant stain and PCR for M. tuberculosis were reported as positive. He was restarted on anti tubercuosis treatment and the abscess disappeared with time. Discussion: Pediatric EN is very uncommon. Since chest x-ray examinations are not always useful, high suspicion and further evaluation may be necessary for diagnosis. Keywords: Child, empyema necessitatis, M tuberculosis [P-149] Wilms tumor with Neurofibromatosis; A rare case report Melih Er1, Elvan Çağlar Çıtak1, Erdem Ak1, Simge Kaya2 1 Mersin University Medicine Faculty, Department of Pediatrics, Mersin, Turkey 2 Mersin Maternity and Child Health Hospital Department of Pediatrics, Mersin, Turkey Introduction And AİM: Wilms’tumoristhemostcommonprimaryrenaltumorinchildhood.80%ofthecasesareseenbetween15yearsofage. Somecongenitalanomaliesandotherdiseasesseen- moreofteninpatientswithWilmstumor:Neurofibromatosis1,BeckwithWidemannsyndrome,DanysDrashsyndrome,Perlmansyndrome,WAGRsyndromeetc. TheriskofmalignancyisincreasedinpatientswithNF1thaninthegeneralpopulation.Themalignantperipheralnevresheathtumorsaremostcommon. Meningioma,ependymoma,Wilms’tumor,rhabdomyosarcoma,neuroblastoma,medullary thyroid carcinoma and melanoma can also seen in these patients.We are present a case of Wilms tumor with a rare involvemet of the posterior bladder in our patient diagnosed with neurofibromatosis Case Report: There was weakness and swelling in the abdomen, especially the right side for 20 days in a patients with neurofibromatosis. The abdominal ultrasound was reported as it was observed a heterogeneous, hypoecoic and hypervascular mass lesion that 12.5x10 cm in size, including cystic degeneration in the middle and have solid component. The multifocal lesions thought to cause diffuse infiltration behind the right kidney and bladder and solid nodular malignant mesenchymal tumors like metastasis in the lung parenchyma has been identified in thoracic and abdominal computer-tomography (CT) scans. The findings obtained by biopsy samples from different regions were reported as being primarily compatible with Wilms’ tumor.The mass was trying to removed as possible as by laparotomy performed by pediatric surgeons, but it could not be removed because there is a total invasion,biopsy was taken and processing was ended. It was determined that the lesion defined behind right kidney and bladder was shrank comparing with older tests after the chemotherapy. Discussion: Ito F. et al. Reported that a 2-year-old male with neurofibromatosis who had a Wilms tumor of the right kidney and an ipsilateral adrenal ganglioneuroblastoma in 1997. Shvartsbeyn et al. reported a case report in 2011. The authors report the first case of a Wilms tumor (WT) with diffuse anaplasia metastatic to the brain in a 13-year-old girl with a history of neurofibromatosis Type 1.As can be understood from these studies in the literature, Neurofibromatosis can accompany each other with Wilms tumor very infrequently. Keywords: Neurofibromatosis, Wilms [P-150] Brucellosis case presented with arthritis on elbow Melike Irem Petan, Hatice Nilgun Selçuk Duru, Funda Kökali Haseki Eğitim ve Araştırma Hastanesi Çocuk Sağlığı ve Hastalıkları Kliniği, İstanbul Introduction: Brucellosis is an infectious disease can spread by contact with infected animals, consuming fresh milk and its products, inhalation of infected droplets. Disease can occur as acute onset fever, splenomegaly, night sweat, joint pain and also some variety of diseases characterised by atypical signs and symptoms that mimic insidious rheumatic or psychosocial tables.Locomotor system complication in children is mostly seen as septic and reactive arthritis.Especially knee and ankle joints are - 79 - involved.Sacroileitis is rare in children.Here we submit a brucellosis case presented with swelling, redness and movement restriction on elbow and initially thought to be a rheumatologic disease or septic arthritis, to draw attention to different involvement of the disease. Case: 15 years old boy was admitted to emergency department with redness, swelling and movement restriction on left elbow.Detailed history showed that he had complaint of migratory arthralgia for 2 months and his family also had same complaints.They had history of eating cheese fermented underground for 6 months in Bitlis.On physical examination there was no other signs.Laboratory and screening tests results were, crp 111 mg/l(0-3), esr 71 mm/hour, wbc 6,2(10e9/ ul), ana positive, brucella IgM positive(>23,6), brucella IgG positive(>17.5), wright test positive in 1/1280 titre,liver size 170 mm(>95p), spleen size 155 mm (>95p).Effusion in elbow joint detected in direct x-ray and mrı and joint puncture was done by consulting orthopedics.Puncture sample was PNL dominant.Patient was diagnosed brucellosis and started doxocyxlin, rifampicine, streptomycin treatment.Streptomycin treatment completed to 14 days.Doxocyxline, rifampicine treatment completed to 6 weeks. Result: Arthritis is seen %10-40 in brucellosis osteoarthricular involvement and especially knee, hip and ankle joint involved.Our country is an endemic region of brucellosis.It is not just animal husbandary sector and also those who consume products derived from the animals are at risk.Therefore arthritis etiology and differential diagnosis of brucellosis should be considered. Keywords: brucellosis, arthritis, elbow [P-151] Bronchiolitis Obliterans In A Patient Require Frequent Nebulizing Treatment and Early Steroid Treatment Melike Irem Petan, Hatice Nilgun Selçuk Duru, Funda Kökali Haseki Research and Training Hospital Pediatrics Department, Istanbul, Turkey Introduction: Bronchiolitis obliterans is characterized by persistant symptoms of obstructive lung disease after an acute bronchiolar injury. It is a rare disease in childhood occurring as a complication of mostly viral(adenoviral) lower respiratory tract infections.Most of misdiagnosed patients require intensive care and mechanical ventilation.Here we submit a bronciolitis obliterans case to draw attention to importance of differential diagnosis in patients who have recurrent bronchiolitis symptoms and starting early treatment that affects prognosis of the disease, number of hospitalization and requirement of picu. Case: 5 years old girl was admitted to emergency department with respiratory distress and tachypnea. She had history of such episodes and hospitalizations for several times.On physical examination, there were diffuse expirium elongation and sibilant rales but no other findings.Laboratory tests were in normal ranges and no significant signs in chest x-ray.On detailed anamnesis she had an adenoviral respiratory infection.Thorax bt imaging was done to patient who had no benefit from inhalation treatment.Thorax bt showed mosaic pattern and vascular attenuation, so the patient thought to be bronchiolitis obliterans and systemic steroid treatment started.After treatment respiratory signs became normal and inhalation treatment requirement was decreased. Patient was thought to be bronchiolitis obliterans because of no benefit of 10 days lasting nebulizing treatment and excluding of other differential diagnosis such as tuberculosis, cystic fibrosis, infectious and rheumatological diseases. Prophylactic steroid treatment and vaccination for flu after externation, decrease nebulizing treatment requirement. Result: Bronchiolitis obliterans should be considered in patient who has recurrent bronchiliotis symptoms, hospitalizations and early steroid treatment should be started.The use of clinical practice guidelines can standardize care, reduce admissions, manage resources better, and shorten the length of hospital stays without increasing readmissions rate or decreasing family satisfaction. Keywords: bronchiolitis, bronchiolitis obliterans, wheezing [P-152] Delirium due to cyclopentolate eye drop in a child Sinan Oğuz1, Melike Ocak2, Nilden Tuygun1, Emine Polat2, Can Demir Karacan1 1 Ankara Dr. Sami Ulus Maternity, Children Health and Diseases Training and Research Hospital, Pediatric Emergency Department 2 Ankara Dr. Sami Ulus Maternity, Children Health and Diseases Training and Research Hospital, Department of Pediatrics Background: Cyclopentolate is a muscarinic receptor antagonist that is widely used topically in ophthalmology for preoperative evaluation and clinical diagnosis because of its mydriatic and cycloplegic activity. When systemic drug absorption occurs central nervous system symptoms may be seen. Here, a four-year-old girl with hallucinations and experienced difficulty in walking after medication of eye drop containing cyclopentolate was presented. Case Report: A four-year-old girl presented with inability to walk, unstable gait, hallucinations and feeling of fear after fifteen minutes of instillation of cyclopentolate for diagnostic eye examination. Physical examination revealed ataxia and incoherent speech and fix dilated pupils. The rest of the examination was unremarkable. Based on these findings and history of cyclopentolate eye drop usage, cyclopentolate toxicity was diagnosed. Her symptoms gradually resolved over the next eight hours. She was discharged uneventfully after 24 hours of follow-up. Discussion and Conclusion: Physicians should be aware of the uncommon systemic side effects of cyclopentolate that is widely used for ophthalmological examinations. Treatment is essentially symptomatic, and nasolacrimal duct occlusion for 3-4 minutes following instillation of - 80 - the drug may reduce its absorption and systemic side effects. Keywords: Delirium, Cyclopentolate, Pediatric Emergency [P-153] Intussusception Presenting with Shock Sinan Oğuz1, Şakire Başer2, Burak Ardıçlı3, Nilden Tuygun1, Melike Ocak2, Can Demir Karacan1 1 Ankara Dr. Sami Ulus Maternity, Children Health and Diseases Training and Research Hospital, Pediatric Emergency Department 2 Ankara Dr. Sami Ulus Maternity, Children Health and Diseases Training and Research Hospital, Department of Pediatrics 3 Ankara Dr. Sami Ulus Maternity, Children Health and Diseases Training and Research Hospital, Pediatric Surgery Background: Shock is defined a dynamic and unstable pathophysiologic state characterized by inadequate tissue perfusion. Hypovolemic shock resulting from gastroenteritis is the most common type of pediatric shock. Early diagnosis and determining cause of shock improves the success of treatment. Here an intussusception case admitted to our pediatric emergency department (PED) with shock state was presented. Case Report: Two-years-old girl was admitted with complaints of vomiting and weakness. Vomiting was repeated many times during the day. After two days of diarrhea she had no defecation for the last 24 hours. She was lethargic with body temperature of 37.6 ° C, pulse 220/min, arterial blood pressure of 80/50 and oxygen saturation of 98%. She was diagnosed and treated as compensated shock. Because of abdominal tenderness and distention she underwent ultrasonography. In the lower right quadrant ileoileal intussusceptions was determined. Surgery reduction was performed and she was discharged on the 5th day. Discussion: Intussusception is an invagination of a part of the intestine into itself. It is the most common cause of intestinal obstruction in infancy. The classic triad of pain, a palpable sausage-shaped abdominal mass, and bloody or currant jelly stool is seen in under one third of patients with intussusceptions. If the intussusception is not reduced, the patient becomes progressively weaker and lethargic. Finally, a shock-like state, with fever and peritonitis, may develop. Reduction of an acute intussusception is an emergency procedure and should be performed immediately after diagnosis. Conclusion: Children with intussusception can be present with classic symptoms but also may be present as an acutely ill child with severe abdominal pain, lethargy, dehydration, and shock. Intussusception should be kept in mind in patients presenting with these symptoms or signs. Keywords: Intussusception, Shock, Pediatric Emergency [P-154] Anticholinergic poisoning due to Mangragora Officinarum in a eight years old boy Sinan Oğuz1, Nilden Tuygun1, Fatma Burçin Kurtipek2, Selman Kesici3, Melike Ocak2, Can Demir Karacan1 1 Ankara Dr. Sami Ulus Maternity, Children Health and Diseases Training and Research Hospital, Pediatric Emergency Department 2 Ankara Dr. Sami Ulus Maternity, Children Health and Diseases Training and Research Hospital, Department of Pediatrics 3 Ankara Dr. Sami Ulus Maternity, Children Health and Diseases Training and Research Hospital, Pediatric Intensive Care Unit Background: Poisoning is a significant fraction of pediatric emergency admissions. The cause of poisoning may not be determined in all cases. Here we present a boy who admitted to our pediatric emergency department (PED) with disorientation, bizarre behavior, visual hallucinations, dilated pupils and blurry vision after unknown plant ingestion. Case Report: Eight years old boy was admitted to PED with mumbling speech, dryness of mouth, blurred vision, bizarre behavior and visual hallucinations after ingestion of an unknown plant root. On physical examination; he had normal vital sings with dilated pupils, disorientation and unbalanced walking. Routine laboratory tests and EKG were normal. The patient was considered as anticholinergic poisoning. The plant had a thick, fleshy, forked root and resembled a human figure that is thought to Mangragora Officinarum and had anticholinergic effects. He was hospitalized and had supportive treatment. On follow-up bladder globe developed so urethral catheter inserted. His signs and symptoms improved after two days and he was discharged. Discussion: Ingestion of some plants can produce anticholinergic toxicity because they contain significant concentrations of belladonna alkaloids (hyoscyamine, hyoscine, atropine and scopolamine). Clinical findings of anticholinergic plant ingestion are similar to classic anticholinergic (atropine-like) toxidrome. Flushed, hot, dry skin, dilated pupils and blurry vision, disorientation, bizarre behavior, paranoia, delirium, visual hallucination, seizure, tachycardia, decreased bowel sounds and urinary retention can be seen in anticholinergic toxicity. Conclusion: Children are at risk being exposed to potentially toxic plants, both in the home and outdoors. The majority of these exposures do not lead to clinically important poisoning. However, anticholinergic poisoning should be kept in mind in patients presenting with plant ingestions. Keywords: Anticholinergic poisoning, Mangragora Officinarum, Pediatric Emergency - 81 - [P-155] An Infant Boy with Big Head Merve Erdemir Kula1, Elif Begüm Kılıç1, Türkan Uygur Şahin1, Emel Torun1, Akın Işcan1, Gözde Yeşil2 1 Department of Paediatrics, Faculty of Medicine, Bezmialem Vakıf University, Istanbul, Turkey 2 Department of Medical Genetics, Faculty of Medicine, Bezmialem Vakıf University, Istanbul, Turkey Introduction: Macrocephaly may be the symptom of an underlying congenital, genetic or acquired problem in children. Here we present a ten month old patient who was diagnosed with Vanishing White Matter Disease (VWMD), a hereditary cause of megaloencephalic macrocephaly. Case: Ten months old infant boy was admitted to our outpatient clinic with large head size. His height was 74 cm (50 p), his weight was 9,3 kg (50 p) and his occipitofrontal circumference(OFC) was 51 cm ( > 98 p) which was increased by five cm at last two months, anterior fontanelle was 2 x 2 cm. The patient was delivered by caesarean section with a 35.5 cm (50 p) of head circumference, after a full-term pregnancy. Prenatal history and neurological development of the boy were normal. The baby was able to hold her head up at two months and sit without support at seven months old. He couldn’t walk yet. He was the second child of unrelated parents whom their OFCs were normal. To eveluate the etiology of our patient’s macrocephaly, blood and urine samples were taken and analysed. There were no abnormalities on his neurometabolic tests. His electroencephalogram was normal. Cranial MRI, that was consistent with VWMD, showed the local encephalopathy which extending from periventricular grey matter to subcortical white matter. The diagnose comfirmed with revealing of MLC -1 mutation by genetic analysis. Discussion: As macrocephaly could emerge hereditary, it also could be the consequence of obstructive/ non-obstructive hydrocephalus, anatomic/metabolic megaloencephalopaty or thickening of skull bones (hyperphosphatemia, osteopetrosis, osteogenesis imperfecta, rickets, dysostosis). One of the most prevalent hereditary white matter diseases, VWMD also known as childhood ataxia with central hypomyelination (CACH). To diagnose VWMD, the mutation of MLC gene should be revealed on the 22th cromosome. Keywords: macrocephaly, infant, white matter [P-156] A rare cause of hypoglycemia in infancy: growth hormone deficiency Merve Hazal Yılmaz1, Tanyel Zübarioğlu1, Ertuğrul Kıykım1, Aydilek Çakır2, Çiğdem Aktuğlu Zeybek1 1 Istanbul University Cerrahpaşa Medical Faculty Department of Pediatrics Division of Nutrition and Metabolism, Istanbul, Turkey 2 Istanbul University Cerrahpaşa Medical Faculty Department of Pediatrics Division of Endocrinology, Istanbul, Turket Growth hormone deficiency is an autosomal recessive inherited disorder characterized by growth retardation. In the early infancy period the only symptom of growth hormone deficiency can be refractory hypoglycemia. It is important to consider growth hormone deficiency in differential diagnosis of hypoglycemia since untreated cases can be life threatening Here a 4-month old female patient who is presented with hypoglycemia and diagnosed as growth hormone deficiency is reported. 4 month-old female patient was referred to our outpatient clinic because of refractory hypoglycemia with a suspicion of fructose intolerance. Initial metabolic investigations including plasma lactate, ammonia, uric acid levels, liver and renal functions, muscle enzymes were found normal. As the laboratory findings of the patient did not support the metabolic disorders and complaint of patient persisted despite the fructose-free diet, initial diagnosis was excluded. In the further investigations, tests for disorders of carbohydrate, aminoacid, fatty acid metabolisms and hyperinsulinemia results were normal. For the differential diagnosis of endocrinologic disorders hormon profile examinations were performed. As the basal growth hormone levels were found to be low during hypoglycemia attack; ACTH loading test and L-DOPA loading tests were made. As sufficient growth hormone increase was not observed, diagnosis of growth hormone deficiency was made. After initiation of As growth hormone replacement therapy, hypoglycemia attacks ceased. Children with growth hormone deficiency may be short at birth, but their lengths are usually within normal limits especially during infancy. In the absence of growth retardation refractory hypoglycemia must be an alerting sign for the phisician. Growth hormone levels should be assessed in any baby with severe or symptomatic hypoglycaemia in whom another cause is not obvious. Keywords: growth hormone, hypoglycemia, infant [P-157] A rare disorder seen in patients diagnosed with cholestasis: Primary bile acid synthesis disorders Merve Nur Hepokur1, Banu Bal Çermik2, Selim Gökçe2 1 Bezmialem University Hospital, Clinics of Pediatrics, İstanbul 2 Bezmialem University Hospital, Clinics of Pediatric Gastroentelogy, İstanbul Primary bile acid synthesis disorders (BASDs) are a group of rare metabolic disorders characterized by defects in the synthesis of bile acids. Overall prevalence is estimated to be around 1-9/1.000.000 for overall BASDs. 3-betahydroxy-delta-5-c27-steroid dehydrogenase deficiency is believed to be the most common form of BASDs. Its deficiency is caused by mutations of the HSD3B7 gene on short arm of chromosome 16 (16p11.2). Clinically; cholestasis, hepatocellular damage, malabsorption, lack of fat-soluble vitamins and cirrhosis can be seen. It can be distinguished from other forms of cholestatic liver diseases in childhood by normal GGT and serum bile acids level and absence of itching. If untreated, progressive liver disease occurs. Diagnosis is based on urine and serum bile acid analysis. Herein, we presented a 19 month-old choletatic male infant without itching. - 82 - He had normal GGT and serum bile acids. Primary bile acid synthesis disorders was eventually diagnosed by demonstration of atypical bile acids in urine. Keywords: cholestasis, bile acids [P-158] Hipotalamic-Pituitary-Adrenal axis supression in children with asthma Merve Şakar1, Şebnem Özdoğan2, Ahmet Uçar3, Ayşenur Kaya4, Sermin Özcan1 1 Department of Pediatrics, Sisli Hamidiye Etfal Search and Training Hospital, Istanbul, Turkey 2 Department of Pediatric Pulmonolgy, Sisli Hamidiye Etfal Search and Training Hospital, Istanbul, Turkey 3 Department of Pediatric Endocrinology, Sisli Hamidiye Etfal Search and Training Hospital, Istanbul, Turkey 4 Department of Pediatric Allegy and Immunology, Sisli Hamidiye Etfal Search and Training Hospital, Istanbul, Turkey Background&Aims: Hypothalamic-pituitary-adrenal axis (HPAS) suppression is among the most important potential adverse effect of ICS. In this study we aim to assess the prevalence and predictive factors for HPAS in children with persistent asthma who has been on daily ICS. Methods: Clinical features of HPAS, steroid dose, and symptom control were documented. Adrenal suppression was measured by morning cortisol levels and confirmed by low-dose adrenocorticotropic hormone stimulating test. Results: A total of 80 children aged 6-18 years old participated. Mean age was 10.24±2.61 years. Two children had HPA axis suppression. Both children with HPA axis suppression were on medium dose ICS. None of the children had clinical features of HPA axis suppression. Conclusions: The clinical significance of effects of ICS on adrenal function still remains to be established. Assessment in large number of patients is ongoing to determine the prevalence and predictive factors of HPAS in children with asthma. Keywords: adrenal supression, asthma, pediatric [P-159] A Patient with systemic involvement: Is tuberculosis the mere cause? Mine Yüksel1, Ahmet Hakan Gedik2, Erkan Çakır2, Nurhan Aruçi Kasap1, Fatma Özgüç1 1 Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Bezmialem Vakıf Üniversitesi, İstanbul, Türkiye 2 Çocuk Göğüs Hastalıkları Bilim Dalı, Bezmialem Vakıf Üniversitesi, İstanbul, Türkiye Background: Childhood tuberculosis (TB) can present in various forms. A rare case of TB with systemic involvements with no detected underlying condition is being presented here. antituberculosis treatment due to mediastinal 1.5 cm lymphadenopathies seen in computed tomography (CT) of the lungs and treatment resistant infiltration in the upper right side seen in pulmonary x-ray when she had presented with cough and sweating 7 years ago (no TB contact, PPD:0 mm, microbiology negative). Hematologic and rheumatologic examinations performed for concomitant leucopenia yielded normal results. Treatment lead to reduction in the lesion and the treatment was discontinued at month 4 by the family. With persisting leucopenia one year later, the lesion returned to its previous sizes, as seen with the pulmonary x-ray taken for night sweatings. With sedimentation 65 mm/h, PPD 15 mm and leucopenia (WBC:4100), the patient was referred to our clinic. Bronchoscopy demonstrated narrowing of apical segment at the upper right segment and disturbed mucosal structure which was accompanied by tracheal bronchus. With transthoracic biopsy showing intraalveolar fibrosis and chronic inflammation, TB organizing pneumonia was considered and anti-tuberculosis and steroid treatment was initiated. Her lesion remitted completely with treatment but she had intermitted leucopenia episodes. 3 years later, she had cough for which pulmonary x-ray was taken and showed recurrence of the lesion in the same region. With pulmonary CT, there was a mass blocking the tracheal bronchus. Biopsy taken with flexible bronchoscopy showed Mycobacterium tuberculosis complex and the patient was started on anti-tb treatment and the lesion disappeared completely at month 2 of treatment Conclusions: Tuberculosis is still a major healthcare problem in our country. In this case, the patient was presented to you as she involved a rare presentation. Keywords: Childhood tuberculosis, organizing pneumonia, systemic involvement [P-160] Regulatory T cells and insulin resistance within the triangle of obesity, asthma and liver diseases Mustafa Metin Donma1, Muhammet Demirkol1, Burcin Nalbantoglu1, Orkide Donma2 1 Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey 2 Department of Medical Biochemistry, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey Background&Aims: Obesity and associated chronic diseases such as asthma and cancer are known with their inflammatory aspects. The impairment of the ability of regulatory T cells (Tregs) to suppress inflammatory responses may lead to obesity-associated inflammation. Obesity-associated diseases are critical risk factors for liver diseases. Effects of obesity on respiratory system appear to play roles in childhood asthma. Hepatotropic viruses also affect lungs as well as respiratory system. Methods: A flow cytometric analysis of T cell subpopulations should be performed in peripheral blood mononuclear cells. Homeostatic Model Assessment for Insulin Resistance was used to determine insulin sensitivity. Case: The 12-year-old female patient was started on Results: Reduction in Tregs may initiate inflammatory - 83 - processes and cause obesity. Obesity in childhood increases the risk of primary liver cancer in adults. Significantly reduced Tregs were detected in obese, asthmatic and obese asthmatic children. Tregs was also reduced during acute hepatitis A. Decreased Tregs lead to reduced suppressive activity and result in liver injury. Inflammation may contribute to the development of insulin resistance (IR). Hyperinsulinemia may impair the ability of Tregs to suppress inflammatory responses and may lead to the development of obesityassociated inflammation. Obese patients with IR display significantly decreased Tregs. Tregs possess a potential therapeutic value to improve IR by limiting inflammation. Visceral obesity enhances hepatitis C virus-induced IR and infection associated with obesity promotes hepatocellular carcinoma development. Conclusions: Interactions among hepatitis, obesity and asthma characterized by chronic and systemic inflammation are quite important. Many bidirectional interactions might contribute to the progression to obese state and then to steatohepatitis and eventually fibrosis, which may be combined with asthma. Common mechanisms and synergy among them are interesting and quite complicated. This needs further investigation. Keywords: Regulatory T cells, insulin resistance, obesity [P-161] Gender difference in association with insulin levels and parental body mass index in underweight children Mustafa Metin Donma1, Muhammet Demirkol1, Burçin Nalbantoğlu1, Birol Topçu2, Orkide Donma3 1 Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey 2 Department of Biostatistics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey 3 Department of Medical Biochemistry, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey Background&Aims: Malnutrition (MN) is one of the most important causes of child mortality alone. The investigations related to the evaluation of abnormal glucose homeostasis, hyperinsulinemia and insulin resistance in malnourished children are extremely limited. The aim of this study is to investigate the availability of the anthropometric measurements, ratios based on them, and insulin sensitivity indices for the evaluation of underweight (UW) children. Methods: Based upon WHO-BMI criteria, of 119 children, 48 were underweight (UW) and 71 were normal weight (NW). The children within 85th – 15th percentiles were evaluated as NW and those below 15th percentiles were included in UW groups. Study protocol was approved by the Ethics Committee and informed consent was taken from the parents prior to study. Anthropometric measurements were recorded. Ratios based upon them as well as insulin sensitivity indices were calculated. The analyses of the body fat were performed. PASW 18 was used for statistical analyses. Results: Significant differences were observed in height:2-to-waist circumference and height:2-to- hip circumference ratios of the children (p<=0.05). Strong correlations were observed between insulin levels and maternal as well as paternal BMIs in UW girls and boys, respectively (p<=0.01). There were strong correlations between HOMA levels and maternal as well as paternal BMIs in UW girls and boys, respectively (p<=0.01). Conclusions: It was shown that togetherness of the mother-her daughter and father-his son, socioeconomic and cultural values shared within the family may all have important effects on intergenerational continuity of the poor eating habits. The important correlations found between insulin as well as HOMA levels and parental BMIs of UW children seem to contribute to the investigations performed on intergenerational transmission. Keywords: Gender difference, insulin levels, underweight children [P-162] Evaluation of the insulin sensitivity indices in accordance with the cut off points in morbid obese boys Mustafa Metin Donma1, Muhammet Demirkol1, Burçin Nalbantoğlu1, Birol Topçu2, Orkide Donma3 1 Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey 2 Department of Biostatistics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey 3 Department of Medical Biochemistry, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey Background&Aims: Obesity is a growing health problem in the world. Especially increasing rate of morbid obesity accounts for serious health problems during childhood. The aim of this study is to evaluate anthropometric measurements and their ratios along with the surrogate indices of insulin sensitivity in morbid obese (MO) boys. Methods: Seventy five MO boys, aged 10.1±2.2 years, participated into the study. Those in the >99 percentile were considered as MO based upon WHO criteria. Written informed consent was obtained from the parents and the study protocol was approved by the Ethics Committee. Anthropometric measurements were carried out. Ratios and insulin sensitivity index (ISI) values were calculated. Children were grouped based on the frequently used cutoff points as well as medians and arithmetic means: The values were designed as below and above 2.0, 2.43, 2.5 and 3.16 for HOMA-IR; 6.0, 7.0, 9.42 and 15.24 for FGIR; 0.328, 0.344, 0.356 and 0.357 for QUICKI. PASW 18 statistical package program was used to evaluate the data. Results: In the evaluation of MO boys, head Circumference (C) /neck C, HOMA/BMI, log HOMA, FMI, Index I and Index II were statistically significant upon evaluation of four different values for each ISI. Especially head C/neck C, Index-I and Index-II were quite significant in evaluation of these parameters according to the values below and above 2.5, 15.24, 0.356 for HOMA-IR, FGIR and QUICKI, respectively (p<=0.01). Conclusions: Evaluation of laboratory criteria is an important approach for upgrading the quality of life of - 84 - children. We concluded that head C/neck C, Index-I and Index-II are important and three new valuable indices for the evaluation of MO boys, when considered based upon ISI indices. Keywords: Insulin sensitivity indices, morbid obese boys [P-163] The positive family history of hypertension and coronary heart diseases among grandparents as a risk factor for childhood obesity Mustafa Metin Donma1, Muhammet Demirkol1, Burçin Nalbantoğlu1, Orkide Donma2 1 Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey 2 Department of Medical Biochemistry, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey Background&Aims: Hypertension (HT) is one of the risk factors of obesity. Its association with coronary heart diseases (CHDs) is also well known. Living with chronic HT can be stressful for those with the diagnosis and their families. Obesity is rapidly increasing during childhood. In this study, the association between HT as well as CHDs in grandparents and obesity in children was investigated. Methods: A total of 118 children, aged 2-14 years, were included into the scope of the study. The body mass indices (BMIs) of seventy-eight were above 95 percentile criteria defined by WHO. Another forty children, whose BMIs are within normal ranges, constituted the control group. The finding of being hypertensive was investigated in grandparents [maternal grandmother (MGM), paternal grandmother (PGM), paternal grandfather (PGF), maternal grandfather (MGF)]. SPSS was used for statistical analyses. Results: In obese group, HT was observed in at least one of the grandparents of 47 out of 78 children (60%). In the control group, this ratio was 14 out of 40 (35%). A statistically significant difference was observed between the groups (p<=0.05). In the obese group, the percentages of hypertensive MGM, PGM, PGF and MGF were 27.5%, 27.5%, 15% and 10%, respectively. In the control group, these values were found as 22.5%, 10%, 7.5% and 5%, respectively. Out of 47 hypertensive grandparents, 39 suffered also from CHDs in the obese group. The frequency of the coincidence of both HT and CHDs was 83% and 57% in obese and control group, respectively (p<=0.05). Conclusions: The preponderance of the grandparental female gender was noted in obese group (55% vs 32.5%). This finding may be confirmed by the future genetic studies. Keywords: Family history, hypertension, childhood obesity [P-164] Application of surfactant in a child case with acute respiratory distress syndrome due to bronchopneumonia Kazım Küçüktaşçı1, Duygu Taşkaldıran2, Nedim Samancı2, Özgür Kızılca3, Muhammet Demirkol2, Nuriye Ece Mintaş2, Hilal Balcı2, Zeynep Ersöz Güngör2 1 Clinic of Paediatrics, Tekirdag State Hospital, Tekirdag, Turkey 2 Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey 3 Division of Paediatric Cardiology, Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey Introduction: Acute respiratory distress syndrome (ARDS) has an incidence of 8.5-16 cases/1,000 pediatric intensive care unit admissions. Primary lung disorders that cause ARDS include pneumonia, aspiration, inhalation injuries, near-drowning and contusions from trauma. These disorders injure alveolar epithelium and increase permeability of the alveolar-capillary barrier. Activity of surfactant is impaired in ARDS due to decreased production, recurrent alveolar collaps during ventilation, and the last and the most importantly due to the dysfunction caused by plasma proteins. Surfactant therapy is aimed to improve gas exchange and protect from the injury caused by the ventilator. In this report, we presented a child case with ARDS due to bronchopneumonia, who benefitted from surfactant therapy. Case: 11 month-old female patient who has neurological sequelae due to meningitis in the newborn period was hospitalized with the diagnosis of bronchopneumonia. Ampicillin-sulbactam, cefotaxime, methylprednisolone, salbutamol, fluid and oxygen with nasal mask were began as treatment. Clinical status of the patient did not improve, and fever occurred. On the 4th day, antibiotherapy was changed to vancomycin and meropenem. On the 6th day of admission, patient was intubated (P-SIMV mode, PIP:28cmH2O, PEEP:7cmH2O, Rate:40/min, FiO2:1.00). SaO2 was around 80-90%. pH:7.10, pCO2:113mmHg, HCO3:35mmol/L, PaO2:67mmHg were detected in the blood gas, and PaO2/FiO2 was 67. Chest radiograph revealed bilateral edema. Echocardiography showed normal pulmonary artery pressure, and there was no heart failure. Patient with suspected primary ARDS due to severe bronchopneumonia was treated with intratracheal surfactant (survanta®) therapy in two doses of 4cc/kg with an interval of 24 hours. After surfactant therapy, SaO2 rised in a short time. Patient was intubated for thirteen days. Antibiotherapy was stopped at 21st day. On the 24th day of admission, she was discharged with cure. Conclusion: We wanted to emphasize that application of surfactant may be beneficial in patients with ARDS by correcting oxygenation. Keywords: Acute respiratory distress syndrome, bronchopneumonia, surfactant - 85 - [P-165] [P-166] A child case of retropharyngeal abscess due to Stenotrophomonas maltophilia after a penetrating trauma Two cases with dystonia due to risperidone intake Muhammet Demirkol1, Özgür Kızılca2, Zeynep Ersöz Güngör1, Nedim Samancı1, Kazım Küçüktaşçı3, Nuriye Ece Mintaş1, Duygu Taşkaldıran1, Hilal Balcı1 1 Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey 2 Division of Paediatric Cardiology, Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey 3 Clinic of Paediatrics, Tekirdag State Hospital, Tekirdag, Turkey Introduction: Retropharyngeal abscess (RPA) is an acute infection of the lymph nodes between the rear wall of the pharynx and the prevertebral fascia. Upper respiratory tract infections and local trauma take role in the etiology. Here we presented a case with RPA developed after a penetrating trauma to the oral mucosa with a pen. Case: 17-month-old male patient was admitted with complaints of fever lasting for 2 days, swelling in the neck, and difficulty in swallowing. He had an history of an oral trauma with a pen 3 days ago. In the first examination he had restlessness, hypersalivation and mild hyperextension of the neck. His AT:38.9°C, HR:137beats/min, RR:25/ min, BP:94/56mmHg. His neck was significantly swollen and he had bilateral multipl lymphadenomegalies. Other systems were normal. His Hb:8.9g/dL, Hct:29.3%, MCV:76fl, WBC:25400/mm3, Plt:510000/mm3, Sed. Rate:101mm/h, CRP:89.8mg/L. In peripheral blood smear 75% PMNL and toxic granulation were determined. Other laboratory tests were within normal limits. Neck ultrasonography revealed bilateral cervical and submandibular lymphadenomegalies the largest of which is 27x11mm. Because patient had history of penetrating trauma, he was consulted to ENT and hospitalized with treatment of 100mg/kg/day iv ceftriaxone and 30mg/ kg/day iv clindamycin. Defervescence occurred and lymphadenomegalies became smaller on the 4th day of treatment. But on the 6th day, fever recurred and stridor became evident. Antibiotherapy was changed to 40mg/ kg/day vancomycin and 90mg/kg/day meropenem. On the 7th day, stridor increased and oxygen desaturation occurred and patient was intubated. Neck CT revealed RPA formation. Abscess was drained and 100.000 cfu/ ml Stenotrophomonas maltophilia with trimethoprim/ sulfametoxazol (TMP-SMX) sensitivity was isolated. On the 3rd day of iv TMP/SMX, defervescence occurred and patient was extubated on the 8th day. On the 10th day, patient was discharged with cure. Results: Retropharyngeal abscess is rarely seen in childhood, but early diagnosis and treatment is of vital importance. Keywords: Penetrating trauma, retropharyngeal abscess, stenotrophomonas maltophilia Muhammet Demirkol1, Kazım Küçüktaşçı2, Nedim Samancı1, Nuriye Ece Mintaş1, Özgür Kızılca3, Ayşin Nalbantoğlu1, Hilal Balcı1, Duygu Taşkaldıran1, Zeynep Ersöz Güngör1 1 Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey 2 Clinic of Paediatrics, Tekirdag State Hospital, Tekirdag, Turkey 3 Division of Paediatric Cardiology, Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey Introduction: Dystonia is characterized by sustained muscular contractions with twisting and repetitive movements or abnormal postures. It is an extrapyramidal adverse effect mostly seen after exposure to dopamine receptor blockers, including anti-emetics, gastrointestinal promotility agents, antipsychotic drugs. However, acute dystonic reactions may be triggered with all drugs that alter dopamine signaling. Clinical manifestations include oculogyric crises, blepharospasm, complex cervical dystonia, focal limb dystonia, and back arching. Acute dystonic reactions respond dramatically to injections of anticholinergic drugs. Biperidene, procyclidine, benztropine, trihexyphenidyl or diphenhydramine may be used to control reaction. Here, we presented 2 children with dystonic reaction due to risperidone intake. Case-1: 15 years-old female patient was admitted to emergency department due to suicidal attempt with 30 risperidone 1 mg tablets and 20 clomipramin 10 mg dragees. Applications of gastric lavage and activated charcoal were done in period of 60 minutes after the pills had been taken. 12 hours later the pills had been taken, she had an acute dystonic reaction in her upper and lower limbs as semiflexion and extension movements. After 10 mg of intramuscular biperidene, the reaction was under control. In her 96 hours of follow up there was no clinical and laboratory finding. The patient was discharged with cure. Case-2: 10 years-old female patient with known epilepsy that is under control with levetiracetam and risperidone was admitted to the emergency department with complaints of vertical deviation in eyes, extension of neck and uncontrolled flexion-extension movements in upper extremities. With suspicion of dystonia, 5 mg dose of intramuscular biperidene was given to the patient and reaction was under control. After 96 hours of follow up, patient was discharged with cure. Conclusions: As a side effect of risperidone, dystonia has to be kept in mind and families should be informed about this effect. Keywords: Dystonia, risperidone, side effect - 86 - [P-167] [P-168] Spondylodiscitis in an adolescent case Nedim Samancı1, Muhammet Demirkol1, Kazım Küçüktaşçı2, Nuriye Ece Mintaş1, Özgür Kızılca3, Hilal Balcı1, Ayşin Nalbantoğlu1, Duygu Taşkaldıran1, Zeynep Ersöz Güngör1 1 Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey 2 Clinic of Paediatrics, Tekirdag State Hospital, Tekirdag, Turkey 3 Division of Paediatric Cardiology, Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey Introduction: Childhood spondylodiscitis represents a process of infectious conditions of the spine, from discitis to vertebral osteomyelitis with occasional associated soft tissue abscess formation. It often presents as backache, restricted spinal mobility, gait disturbance, refusal to walk and sit, deteriorated general condition, and a need to hold on to objects for support. While the inflammation markers increase slight to moderate degree, blood cultures are usually negative. Although radiographs should show intervertebral disc height narrowing if symptoms have exceeded 10 days, further imaging with MRI is likely to confirm the diagnosis. Because childhood spondylodiscitis is extremely rare in childhood, diagnosis is often established after an extensive delay. Treatment includes antiinflammatory agents, antibiotics and immobilization. Here, we presented an adolescent girl with a diagnosis of spondylodiscitis who was treated successfully. Case: 14 years-old female patient was admitted to outpatient clinic with complaints of fever and lower backache. Physical examination was normal. Her Wbc:7740/mcl (72% neutrophilia), ESR:38mm/ hr, CRP:84mg/L (<10); ASO, RF, Rose Bengal, ANA, Anti-dsDNA, PPD, QuantiferonTB, HLA-B27 were negative. Chest radiograph, abdominal-urinary ultrasonography, echocardiography were normal. Contrast-enhanced lumbosacral MRI showed significant contour derangement, T1-hypointense, T2-hyperintense pathological signal difference and dense contrast enhancement in inferior part of L2 and superior part of L3 vertebrae corpora with narrowing of the distance between both. 99mTc MDP whole body scintigraphy supported the diagnosis of spondylodiscitis suggested in MRI. Vankomisin intravenous, cefotaxime intravenous, and ibuprofen po were started as therapy. On the 5th day of therapy, complaints of the patient were relieved. On the 21st day of therapy, the patient was discharged with treatment of oral fusidic acid and ciprofloxacin in order to be used for 3 weeks. Eventration of the diaphragm as a rare cause of cyanosis in infancy Özgür Kızılca1, Kazım Küçüktaşçı2, Muhammet Demirkol3, Nedim Samancı3, Zeynep Ersöz Güngör3, Hilal Balcı3, Duygu Taşkaldıran3, Ayşin Nalbantoğlu3 1 Division of Paediatric Cardiology, Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey 2 Clinic of Paediatrics, Tekirdag State Hospital, Tekirdag, Turkey 3 Department of Paediatrics, Faculty of Medicine, Namik Kemal University, Tekirdag, Turkey Introduction: Eventration of the diaphragm is rare in childhood. Birth trauma and cardiac surgery are the most common causes. Right-side involvement is more common. Elevated diaphragm with fields of atelectasis in chest radiograph provides the diagnosis. Thorax CT and fluoroscopy also clarify the status. Treatment is support in the beginning. Surgical plication is the treatment of choice in the patients with significant respiratory distress or without spontaneous regression. Here, we presented a patient with cyanosis in whom we detected unilateral eventration of the diaphragm. Case: 6 months-old male patient was admitted to emergency department with diarrhea, vomiting and fever. Cyanosis was realized and pO2 saturation was detected 82-84%. He was hospitalized for further evaluation. His weight: 5,6 kg (<3p), height: 68 cm (10-25p), BP: 88/43 mmHg, RR: 45/min and fingers had clubbing. He had abdominal breathing pattern and decreased breath sounds on the base of right lung. Cardiovascular system and other systems were normal. Routine laboratory tests of CBC, CRP, KFT, LFT and electrolytes were normal. In venous blood gas pH:7.36, PCO2: 48mmHg and bicarbonate: 27mmol/L. Chest radiograph revealed lung opacities on the base of right lung. ECG and echocardiography showed no abnormality. Elevated right diaphragm and basal atelectasis in the right lung were also detected in the chest CT which is done in suspicion of a mass. Nebulised salbutamol and fluticasone with postural drainage were started to the patient as a treatment. His paO2 saturation raised to 92% in room air with treatment. Diaphragmatic paralysis was detected in the fluoroscopic examination of the patient. Then the patient was discharged with a plan of diaphragmatic plication. Result: Eventration of the diaphragm, as a rare disorder, has to be kept in mind in case of cyanosis. Keywords: Cyanosis, diaphragmatic paralysis, eventration of the diaphragm Conclusion: Spondylodiscitis, as a rare disease of childhood, should be kept in mind in differential diagnosis of patients with complaints of fever and backache. Keywords: Adolescent, spondylodiscitis - 87 - [P-169] A case of central nerves system tuberculosis diagnosed by biopsy Murat Sutcu1, Manolya Acar1, Ayper Somer1, Aslı Toros1, Nur Aydınlı2, Mine Çalıskan2, Cicek Bayındır3, Nuran Salman1 1 Istanbul University Istanbul Medical Faculty, Pediatric Infectious Diseases 2 Istanbul University Istanbul Medical Faculty, Pediatric Neurology 3 Istanbul University Istanbul Medical Faculty, Pathology Introduction: Central nervous system tuberculosis is the most serious form of tuberculosis which can be mortal unless treated effectively. Several forms of infectious and non infectious diseases should be eveluated in differential diagnosis. Surgical intervention may be necessary for certain diagnosis in selected cases. Case presentation: Six year old child with history of herpes encephalitis, presented with headache, vomiting and confusion. Physical examination and initial laboratory evaluation were normal. Cranial MRI revealed hypodense edematous lesion with shifting on right temporoparietooccipital lobe. Necrotizing granulomatous inflammation and acid resistant stain positive bacilli were identified from biyopsy material. Antituberculous therapy and corticosteroid treatment were initiated. After 18 months of antituberculous treatment, child recovered well with normal neuromotor development. Conclusion: In the cases when differential diagnosis is impossible with clinical and radiological findings, surgical intervention may be obligatory. Keywords: Biopsy, child, central nervous system tuberculosis [P-170] Electrical storm in an adolescent with arrhythmogenic right ventricle cardiomyopathy resulted in cardiac transplantation Hayrettin Hakan Aykan1, Mustafa Gülgün2, Ilker Ertuğrul1, Tevfik Karagöz1 1 Hacettepe University, Pediatric Cardiology, Ankara/ Turkey 2 Gulhane Military Medical Academy, Pediatric Cardiology, Ankara/Turkey Arrhythmogenic right ventricle dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterized by fibrofatty replacement of the right and less frequently left ventricle. Ventricular arrhythmias requiring implantable cardioverter defibrillator (ICD) are common in patients with ARVD/C. Electrical storm (>=3 life-threatening ventricular arrhythmia within 24-hour period) resulting in ICD discharges is a major cause of morbidity and mortality. We had experience of a case who 15 year-old boy presented with chest pain and decreased exercise capasity. He had been undergone surgical closure for atrial septal defect and complicated with ventricular tachycardia/fibrillation (VT/VF) postoperatively at 11 years-old. No history of congenital heart defect in family and syncope were found. Premature ventricular contractions were determined occasionally in 24-hours Holter monitoring. Echocardiography demonstrated dilatation of right ventricle and right ventricle outflow tract. The dilated infundibulum and increasing in the right and left ventricular trabeculation were demonstrated in the cardiac angiography and magnetic resonance imaging. Non-sustained monomorphic VT (260 beats/ minute) was induced during electrophysiologic study. He was diagnosed as ARVD/C and ICD was implanted for primary prevention. Two years later, the episodes of VT/VF was occurred 35 times within one month. He was started on amiodarone and sotalol and the radiofrequency ablation was performed. Two months later, the electrial storm repeated again and the patient was arrested in a short time. He was immediately connected to the pump after cardiac resuscitation and undergone the cardiac transplantation one day later. He has been on follow up with no symptom for three year. ICD seems indispensable and the ablation of VT can be considered for reducing the side effects of the drugs and prolonging a ICD battery life in ARVD/C currently. The management of electrical storm should be individualized for each patient and the treatment may indicate extracorporal membrane oxygenation and cardiac transplantation. Keywords: electrical storm, cardiomyopathy, transplantation [P-171] Kawasaki disease shock syndrome: Case Report Ayşegül Dogan Demir, Selçuk Uzuner, Ufuk Erenberk, Mustafa Oğur, Özden Türel, Yılmaz Yozgat, Mebrure Yazıcı, Nurettin Onur Kutlu Pediatrics, Bezmialem Vakif University, Istanbul, Turkey Background: Kawasaki disease is a rare self-limited systemic pediatric vasculitis and results in coronary artery aneurysms in 20% of untreated patient. During acute phase of illness, hemodynamic instability and shock is uncommon. Kawasaki disease shock syndrome (KDSS) was recently described is associated with moresevere laboratory markers of inflammation and mitral regurgitation, prolonged myocardial dysfunction and shock. Case: We report twelve-year-old who presented with mumps and a toxic shock-like syndrome was treated with fluid resuscitation and vasoactive infusion. Finally our case was diagnosis with Kawasaki disease shock syndrome when right giant coronary artery aneurysms were found on subsequently echocardiographic examination Result: KDSS should be considered in all children have prolonged fever, most- overt systolic and diastolic dysfunction, and shock. pediatricians assess for coronary artery abnormalities with subsequently echocardiographic examination in all children have severe myocardial dysfunction, prolonged fever and shock syndrome. Keywords: Kawasaki disease, echocardiographic examination - 88 - [P-172] [P-173] Mustafa Taha Özkul1, Melis Köse2, Mehtap Kağnıcı2, Cahit Barış Erdur1, Erhan Özbek1, Ferah Genel1 1 Pediatri Anabilim Dalı,Dr.Behcet Uz Cocuk Hastaliklari Hastanesi Egitim ve Arastirma Hastanesi, Izmir, Turkiye 2 Cocuk Metabolizma Hastaliklari Bilim Dalı, Dr.Behcet Uz Cocuk Hastaliklari Hastanesi Egitim ve Arastirma Hastanesi, Izmir, Turkiye Background&Aims: Citrin deficiency, which is encoded by SLC25A13 gene, is a disorder with two phenotypes: neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), and adult onset type II citrullinaemia (CTLN2). NICCD is an autosomal recessive metabolic disorder characterized with intrahepatic cholestasis and increased serum citrulline. Symptoms resolve within the first year of life mostly without special treatment. However, some patients may develop more severe symptoms of CTLN2 like neuropsychiatric symptoms and hyperammonemia on adulthood. The objective of this study is to report the case of a 2.5 months old boy with NICCD, focusing on its diagnosis and treatment. Methods: A 40-week gestation male, with a birth weight of 3200 grams, was born after an eventful pregnancy with cesarean delivery, second child of healthy nonconsanguineous couple. He was exclusively breastfed. He was admitted to hospital for prolonged jaundice. He had jaundice, pallor and hepatomegaly. Acholic stool was negative. Total bilirubin was 11.6 mg/dl, direct bilirubin was 4.6 mg/dl, serum α-fetoprotein level was 51643.62 ng/mL. Infectious causes of neonatal jaundice were negative. Abdominal ultrasonography was normal. Metabolic evaluation disclosed elevation of blood lactate, blood ammonia and citrulline, methionine, threonine, arginine and lysine among plasma aminoacids. Serum total galactose, free galactose and galactose 1-p levels were found elevated with normal galactose 1-p uridil transferase activity. These findings and mutation analysis of SLC25A13 gene confirmed NICCD. Patient was treated with high protein diet, fat-soluble vitamins, ursodeoxycholic acid. Mustafa Torehan Aslan1, Benhur Sirvan Cetin2, Taylan Celik2, Enes Sali2, Solmaz Celebi2, Mustafa Hacimustafaoglu2 1 Uludag University Medical Faculty Department of Paediatrics, Bursa/TURKEY 2 Uludag University Medical Faculty Department of Paediatric Infectious Diseases, Bursa/TURKEY Introduction: Childhood pneumonia, especially in developing nations is one of the most important morbidity and mortality causes. Round pneumonia (RP), a subgroup of pneumonia, is characterised by round increased opacities in the chest X-Ray. Our case is presented because RP accompanied by leukemoid reaction is rare and the presence of malignity in differential diagnosis. A rare cause of neonatal cholestasis;citrin deficiency Results: Patient has shown improvement in clinical follow-up and observed the progressive normalization of liver function tests and aminoacid profile with in few months. Conclusions: In neonatal cholestasis, early recognition of NICCD is fundamental to apply specific diagnostic tools and appropriate dietary treatment, giving the possibility of a close monitoring of patients in follow-up care and ways to prevent the development of CTLN2. Keywords: Citrin deficiency, Citrullinemia, Neonatal intrahepatic cholestasis A Round Pneumonia Case with Leukemoid Reaction Mimicking Lung Malignity Case: A 3 year 7 months male patient with fever, cough and difficulty in breathing complaints that started two weeks ago and did not show improvement after oral antibiotics prescription from a health center, fever and cough increased over the last two days. Physical examination; right mid-lower lung zone localized decreased breathing sounds and widespread crackles, bilateral rough lung sounds. Complete blood count; leukocytosis (53,900/mm³), peripheral smear had 86% neutrophilia, two positive toxic granulation with no blast or atypic cell. Biochemical tests; C-Reactive Protein 9.87mg/dl, Procalcitonin 2.37ng/mL, Erythrocyte Sedimentation Rate 26mm/hour. Chest X-Rays showed right paracardiac increased consolidation and the right lung lower zone had demarcated 24x15mm round, contoured opasity increase. A Thorax Computed Tomography taken for further investigation and differential diagnosis revealed a consolidation region compatible with RP in the lower lobe’s posterio-basal segment of the right lung. Parenteral cefotaxime antibiotherapy was initiated. After three days of parenteral antibiotherapy clinical and laboratory findings showed significant improvement and the patient was discharged with a 14 day oral sequential therapy (Amoxicilin-Clavulanic Asid). After completing the treatment our patient came for out-patient clinic control and showed full improvement in his complaints, laboratory findings and chest X-Ray. Conclusion: Other than RP being a well described radiologic entity, RP accompanied by leukemoid reaction is a rarely seen condition. Radiologically solitary, demarcated mass lesion has malignities in the differential diagnosis. Our case’s first complaints resembled malignity but full improvent with appropriate antibiotherapy left no need for further investigation for differential diagnosis. Keywords: Round Pneumonia, Child, Leukemoid Reaction - 89 - [P-174] A Case with Widespread Herpes Virus Dermatitis and Conjunctivitis Mustafa Torehan Aslan1, Sefika Elmas Bozdemir2, Solmaz Celebi2, Benhur Sirvan Cetin2, Mustafa Hacimustafaoglu2 1 Uludag University Medical Faculty Department of Paediatrics, Bursa/TURKEY 2 Uludag University Medical Faculty Department of Paediatric Infectious Diseases, Bursa/TURKEY Introduction: Conjunctivitis in children is common in pediatric emergency departments. It is characterised with severe tear flow, conjunctival hyperemia, chemosis, watering, itching and pain. Conjunctivitis due to herpes simplex virus (HSV) may be primary infection or recurrence, and is unilateral in 80% of the cases. This report presents a child with bilateral HSV conjunctivitis and dermatitis that showed full lesional improvement after two weeks of systemic and local treatment. Case: A 3 year old male patient, no history of known systemic illness, presented with skin eruptions that started a week ago, first on the eyelids then spread to the whole eye area accompanied with erythema and eye discharge that did not respond to local treatment prescribed by eye specialists. Physical examination revealed widespread vesicular skin lesions with hyperemic ground on the eye area spreading towards the face and purulent flow from both eyes. Other systemic examinations were normal. Full blood count; leucocytes 8170/mm³, haemoglobin 11.6gr/dl, platelet 331000/mm³, C-Reactive Protein 0.47mg/dl. The case was evaluated by the departments of Pediatric Infectious Diseases and Eye Diseases and diagnosed with herpes virus dermatitis and herpes virus conjunctivitis without corneal involvement; intravenous acyclovir, cefuroxime, clindamycin and local acyclovir eye ointment, tobramycin eye drops, ofloxacin eye drops and biborate de soude eyewash treatments were initiated. After 10 days of treatment almost all the lesions dried up and conjunctivitis healed. Conclusion and Discussion: The case was presented in order to attract attention on the rarely seen pediatric bilateral HSV conjunctivitis and dermatitis and the need for systemic treatment. Keywords: Herpes Virus Dermatitis, Child, Conjunctivitis [P-175] a leishmaniasis case present with prolonged fever and hepatosplenomegaly Müferet Ergüven, Doruk Gül, Asena Pınar Sefer, Ismet Düşmez GOZTEPE RESEARCH HOSPITAL ISTANBUL/TURKEY Introduction: Leishmaniasis, is a common name of a clinical syndrome caused by Leishmania specieses which is an obligatory intracellular parasite. Leishmaniasis is an endemic disease in our country and our neighbour countries. Leishmania specieses cause three main clinical form of diseases which are cutenous,mucocutoneous and visceral.Infected sand fly is the vector of leishmaniasis.. Systemic leishmaniasis is a progressive infectious diseases which mainly affect reticuloendotelial system. If the disease treated unproperly,it may cause death. %80 of the cases are younger than 5 years old. Because of the prolonged incubation period and unspecific symptoms; Its hard to diagnose of the disease. In this case, we described a systemic Leishmaniasis present with prolonged fever and hepatosplenomegaly. Case: A 2 years old girl was admitted to a hospital with prolonged fever which was continues about 15 days and unresponsive to antibiotic treatment.The fever was relapsing 2-3 times in a day, reaching to 40 degrees and decreased spontaneously She hadn’t any complain other than fever The physical examination of the patient; she had hepatosplenomegaly about 4-5 cm.The other findings were normal. In laboratory; Hb:8.03g/ dl,Plt:156000,Wbc:4.1,Neu:770,Crp:0.33mg/dl biochemical analysis:normal urinalyse:normal Chest x-ray: normal In abdominal USG;liver and spleen size increased We started broad spectrum antibiotic.In the 72.hour of the antibiotic;the patient still had fever and her hepatosplenomegaly were increased. The laboratory findings;Hb:5.8mg/dl plt:130000,wbc:6700,neu:670,Crp:2.7mg/dl Because of continued fever and pancytopenia; We made bone marrow aspiration Leishmania amastigotes were seen in her bone marrow aspiration material. The patient was diagnosed to Leishmaniases and amphotericin b treatment was started.In the 48. hour of treatment the fever was decreased dramaticaly and the sizes of liver and spleen decreased. At the 10th day of the treatment patient was fully recover and externated from hospital. Keywords: leishmaniasis, prolonged fever, hepatosplenomegaly [P-176] The Etiology of Hypertransaminasemia in Children Filiz Serdaroglu1, Mustafa Akcam2, Tugba Koca2, Selim Dereci2, Nagehan Aslan1 1 Department of Pediatrics, S. Demirel University, Medical School, Isparta, TURKEY 2 Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, Faculty of Medicine, S. Demirel University, Isparta, Turkey Aim: To investigate the etiology and demographic characteristics of children determined with high levels of transaminase in our hospital over a period of two years. Material-Method: Children aged 3 months – 8 years who presented at our hospital between December 2012 and December 2014, either as in-patients or outpatients and were determined with elevated transaminase (ALT>45, AST>50 IU/L) were identified from the hospital records system (MEDULA). Retrospective evaluation was made of these children from the records in respect of demographic characteristics, diagnosis and tools used - 90 - for diagnosis. Results: The study included a total of 281 children who met the study criteria. These comprised 125 (44.5%) females and 156 (55.5%) males with a mean age of 7.01±5.24 years. The most common complaint was weakness (53.4%) and 15.7% had no complaints. In the physical examination the most common findings were jaundice and hepatomegaly and 15% of cases were completely normal. In the etiology, infectious causes (34%) were most common and within these, HAV infection (18.9%) was the leading cause. Drugsrelated causes were determined at a rate of 18.1% and non-alcoholic fatty liver disease (NAFLD) at 11.1%. Idiopathic cases were seen at the rate of 6.4% and all were seen to have returned to normal levels at mean 95 days (range, 7 days- 14 months). The highest levels of transaminase were determined to be caused associated with HAV infection and slightly elevated levels were caused by medications and NAFLD. Conclusion: The results of this study showed that elevated transaminase was caused most often by infections, drug toxicity and NAFLD. Elevated transaminase is often encountered in daily practice and even though it indicates liver disease in the majority of cases it may also be associated with a cause other than the liver and may be seen in completely healthy individuals. Keywords: Children, hepatitis, transaminase [P-177] Early-onset chromhidrosis in an infant Nagehan Aslan, Şenay Akbay, Ali Ayata Department of Pediatrics, Suleyman Demirel University Faculty of Medicine, Isparta, Turkey Chromhidrosis is the production of colored sweat by eccrine or apocrine sweat glands. There have been very few reports of chromhidrosis in the pediatric age group. We report a 4 month old girl with this rare dermatological disorder. A 4-month-old girl presented with a history of staining of her undershirt, especially the axillary region. This coloration noted by parents for last two month. She was born after a normal pregnancy from non-consanguineous parents. She had no history of drug use or illness. She was fed with brest milk. On physical examination it was noticed that her undershirt was bluish colored (Fig.1). The rest of the physical examination was normal. Routine laboratory investigations were normal. Urinary homogentisic acid level was within normal limits. Microbiological examination of her skin revealed normal skin flora. Clinical diagnosis was established. Skin biopsy was not performed Chromhidrosis refers to secretion of colored sweat and was first reported in 1709 by Yonge of Plymouth (1). It has been classified into apocrine, pseudoeccrine, and true eccrine chromhidrosis. Apocrine chromhidrosis refers to the secretion of colored sweat. Apocrine sweat glands are located in the axillae, anogenital skin, areolae and over the skin of the trunk, face and scalp. The color of chromhidrosis may be yellow, green, blue, brown or black, depending on the level of oxidation of lipofuscin secreted in sweat. In conclusion, although apocrine chromhidrosis may develop at any age, it is more often after puberty. The age of the patients reported in the literature range from 9 months [5] to 76 years. Carman et al. report a 9 month patient reported from Turkey. Our patient was chromhidrotic for 2 months. To the best of our knowledge, our 4-month old patient might be the youngest case in the literature. Keywords: Chromhidrosis, colored sweat, 4-month-old [P-178] A Rare Case: Congenital Asymmetric Crying Face Neslihan Özhan1, Ece Gültekin2, Güzide Turanlı2 1 Pediatrics, Faculty of Medicine, Medipol University, Istanbul, Turkey 2 Pediatric Neurology, Faculty of Medicine, Medipol University, Istanbul, Turkey Introduction: Asymmetric crying face is an abnormality caused by congenital hipoplasia or agenesis of unilateral depressor anguli oris muscle. The major finding is the absence or weakness in the outer or lower movement of the corner of mouth during crying or smiling. The other expressions of the facial muscles are normal and the face is symmetric at rest. The major differential diagnosis is central facial paralysis. To avoid invasive diagnostic procedures and potential complications, pediatricians should be consider this congenital abnormality. Case Presentation: This is a newborn presented with asymmetric movement of right corner of the lower lip since birth. The facial asymmetry was more pronounced when she cried. The other facial movements were essentially symmetrical. The rest of the neurological examination was normal including facial nerve function, as determined by frowning forehead, wrinkling, eye closure, nasolabial fold depthness and tearing. Cranial ultrasound was normal and echocardiogram did not reveal any cardiac abnormality. Auditory brainstem response did not indicate any abnormality. Conclusion: Congenital hypoplasia or agenesis of depressor anguli oris muscle is a rare abnormality and it is mainly diagnosed by neurological examination. Although electromyelographic evaluation is not necessary to detect the muscle weakness sometimes it could be beneficial for differential diagnosis. The most common problem is false diagnosis of central facial paralysis in a patient with asymmetric crying face. Cervicofacial, musculoskeletal, respiratory, genitourinary and central nervous system anomalies could be associated with congenital unilateral hypoplasia or agenesis of depressor anguli oris muscle. In our case no other malformation was detected. Combination of clinical suspicion and detailed physical examination for abnormalities in other systems ensures early diagnosis and so prevents from invasive diagnostic procedures in children with asymmetric crying face. Keywords: asymmetric crying face, agenesis of depressor anguli oris muscle - 91 - [P-179] A patient with tuberosclerozis who is tachyarrhythmic without rhabdomyoma Nihat Cem Çelebi1, Aslı Nur Ören1, Ali Oruç1, Leyla Telhan2 1 Istanbul Medipol University, Faculty Of Medicine Department Of Pediatrics 2 Istanbul Medipol University, Faculty Of Medicine Department of Pediatric Intensive Care Tuberosclerosis (TS) is an Autosomal Dominant neurocutenous syndrome, characterised with multipl hamartomatous lesions that are seen in many organs such as skin, brain, eyes, kidneys and heart. This syndrome frequently associated with cortical dysplasia, subependimal nodules, giant cell astrocytoma, retinal astrocystic hamartoma, facial angiofibroma, renal and lung angiolipoma and cardiac rhabdomyoma. Hamartomatous lesions are usually asymptomatic. For this reason,these findings are guide for diagnosing of cases without mental retardation and/or convulsions. Pathognomonic skin findings of tuberous sclerosus are adenoma sebaceum, periungual fibromas, shagreen patches and ash-leaf macules. Cardiac rhabdomyomas are seen in sixty percent of the cases that under age of two. Supraventricular and ventricular tachycardias, A-V blocks may also accompany for cardiac rhabdomyomas. Tuberous sclerosis is usually considered in cases of cardiac arrhytmias that are determined during healthy children follw-up. But however, characteristic skin lesions are generally missed. In this case report, we try to emphasize the importance of physical examination. Keywords: tuberosclerozis, tachyarrhythmia, rhabdomyoma [P-180] A case of hypocalsemic convulsion caused by vit d deficiency Nilay Kadakal1, Ayşegül Doğan Demir1, Ufuk Erenberk1, İlker Tolga Özen2, Türkan Uygur Şahin3 1 Bezmialem Vakıf University,Department of Pediatrics,Istanbul,Turkey 2 Bezmialem Vakıf University,Department of Pediatric Endocrinology,Istanbul,Turkey 3 Bezmialem Vakıf University,Department of Pediatric Neurology,Istanbul,Turkey Background&Aims: Nutritional Rickets is the most important result of vitamin d deficiency in childhood. It typically presents between four months and three years of age and associated with prolonged breastfeeding without vit d supplementation. Vitamin D deficiency presents with medical conditions such as growth disturbance,bone deformities,muscle weakness,hypoplasia of teeth, susceptibility to infections and also can cause serious complications such as hypocalsemic convulsion Nutritional vitamin D deficiency is diagnosed by clinical manifestations and typical laboratory findings such as hypocalcemia,hypophosphatemia,hyperparathyroidism,and low level of 25 hydroxyvitamin D. Methods: A previously healty 10 -months –old -boy has applied to our hospital for generalized tonic –clonic convulsions.The patient had not received any vitamin d supplementation and adequate nutritional intake of calcium and phosphorus after the birth. Laboratuvary test have shown that calcium:5,9 phosphorus:3,5 Alp:829 Pth:412(15-65) 25 OH vit D <4 ng /ml, indicating hypocalcemia,hypophosphatemia,hyperparathyroidism,and low level of 25 hydroxyvitamin D.In physical examination;we have found open and large fontanel,caput quadratum deformity and kraniotabes.Hand-wrist radiograph showed metaphyseal hyperplasia,The patient was diagnosed with nutritional rickets.He was treated with vit D and calcium lactat replacement.After 3 days of hospitalization,the patient was discharged with normal clinical manifestations and laboratory findings Results: Vitamin D is an essential nutrient that plays an important role in calcium homeostasis and bone health. Vitamin D deficiency has been associated with rickets, a disease in which the bone tissue doesn’t properly mineralize, leading to soft bones and skeletal deformities and also can cause serious complications such as hypocalsemic convulsion tetany and larnygospazm. Conclusions: As a result;we present hypocalsemic convulsion in a 10 months old boy with vit d deficiency.This case is an example of serious complications of nutritional rickets as an important public health problem despite vitamin d supplemantation is common in our country. Keywords: hypocalcemia, vit d deficiency, rickets [P-181] An Important Cause Of Persistant Vomiting In Childhood: Superior Mesenteric Artery Syndrome Tufan Kutlu1, Nisa Pekcan2, Hasret Ayyıdız Civan1 1 İstanbul Üniversitesi Cerrahpaşa Tıp Fakültesi Çocuk Gastroenteroloji 2 İstanbul Üiversitesi Cerrahpaşa Tıp Fakültesi Çocuk Sağlığı ve Hastalıkları Superior Mesenteric Artery Syndrome (also known as Wilkie’s syndrome) has many causes including high insertion of the duodenum at the ligament of Treitz a congenitally low origin of the superior mesenteric artery and compression of the duodenum due to peritoneal adhesions as a result of duodenal malrotation. However,the most common cause is thought to be narrowing of the aorta-mesenteric angle causing extrinsic compression of the third part of the duodenum. Herein, a 13 year-old male patient presented with persistant vomiting and 15-kg weight loss during last 8 months who is diagnosed as superior mesenteric artery syndrome is reported. The patient was admitted to our out-patient clinic with severe vomiting and weight loss. The patient had a history of obesity who started a diet programme on his own and lost 15 kilograms of weight during last 8 months. He had complaint of severe vomiting after each meal. His physical examination was unmarkable except cachexia. He did not have abdominal pain or tenderness. In scope of this history we suspected superior mesenteric artery syndrome. We performed double contrast barium passage graphy which showed compression of first, - 92 - second and third part of duedonum. CT-angiography scan had revealed elongation and increase in volume of stomach. Besides dilatation of the first, second and third part of duedenum. Distance and angle between superior mesenteric artery and abdominal aorta was measured as 2 mm and 18 degrees, respectively. These findings were compatible with superior mesenteric artery syndrome. We have recommended to insert a nasojejunal tube, however patient refused this treatment. Conclusion: In general, when signs and symptoms of upper gastrointestinal obstruction are accompanied with rapid weight loss, superior mesenteric artery syndrome should be considered. The most useful diagnostic tool to detect superior mesenteric artery syndome is CT anjiography scanning. Keywords: superior mesenteric artery syndrome, Wilkie syndrome [P-182] Neuro-Behçet’s Disease in Childhood Nurcan Yusufoğlu, Burcu Karadaş, Dilşad Koca, Zehra Esra Önal, Çağatay Nuhoğlu Department of Pediatrics, Haydarpaşa Numune Training and Research Hospital, Istanbul, Turkey Introduction: Behçet’s Disease is a multisystemic disease that commonly presents dermal, ocular, vascular, nervous and joint involvements. The disease course has remissions and attacks, and usually involves recurrent oral aphthous ulcers. Behcet’s disease is most commonly diagnosed between the ages 20-40. It rarely can be diagnosed on early ages. Case: 13-year-old male patient was admitted with a headache that has a duration for a month and decreased visual acuity. The eye exam demonstrated that he had panuveitis, neuroretinitis and bilateral papileodema. He had recurrent oral aphthous ulcers on his history. Right transverse sinus thrombosis had been seen on the cranial Magnetic Resonance Imaging (MRI) and Magnetic Resonance Venography. Antitrombin 3, Protein C, Protein S, Factor V Leiden, Prothrombin 20210, Methylene tetrahydrofolate reductase (MTHFR) mutations has been searched, results were negative. Pathergy test result was positive. HLA-B51 was found as a result of histological evaluation. With the data in hand the patient was diagnosed with ‘Behçet’s Disease’ and he was sent to Children Rheumatology Department for treatment and follow-up. Discussion: Our patient had recurrent oral aphthous ulcers in his history. In our case panuveitis and neuroretinitis were found. Severity of ocular involvement and loss of vision are related to the early onset of the disease and the patient’s gender being male. Vascular involvement may occur at any kind of vein (but venous is the most) that is commonly seen in male patients. Our patient had right transverse sinus thrombosis. Our patient’s pathergy test was positive. Behçet’s Disease has no specific laboratory findings. HLA-B5 and HLA-B51 positivity can be demonstrated. Treatment is symptomatic. Conclusion: Prognosis for Behçet’s Disease is worse in young ages and males. Diagnosis at early stages of the disease and monitoring with multi-disciplinary approach have a great importance for the prognosis of the disease. Keywords: Neuroretinitis, Oral ulcers, Thrombosis [P-183] Fanconi Anemia with VACTERL association Nurhan Aruçi Kasap1, Fatma Betül Çakır2, Gözde Yeşil3, Fatma Özgüç1, Mine Yüksel1 1 Bezmialem Vakıf Üniversity Medical Faculty, Paediatrics, Turkey/İstanbul 2 Bezmialem Vakıf Üniversity Medical Faculty, Paediatric Hematology and Oncology, Turkey/İstanbul 3 Bezmialem Vakıf Üniversity Medical Faculty, Medical Genetics, Turkey/İstanbul Introduction: Fanconi aplastic anemia (FA) is a disease that effects several systems. There is a risk for bone marrow deficiency, physical abnormalities, organ defects and some cancers. At least three of VACTERL syndrome features (vertebral abnormalities, anal atresia, cardiovascular abnormalities, tracheoesophageal fistula, renal abnormalities, extremity defects) are seen in 5% of these patients. An 8-year-old female patient who was diagnosed with FA and determined to have VACTERL association is being presented in this case report. Case Report: The 8-year-old female patient, who presented to our clinic with seizures, was examined and found to have pancytopenia. With history of esophageal atresia, operated tracheoesophageal fistula, operated anal atresia, prenatal hydrocephalus-VP shunt, renal agenesis, bilateral external auditory canal agenesis, patient’s examination demonstrated absence of a thump, clinodactilia, micrognathia, triangular-shaped face, lowset ear, toe abnormalities, labial synechia. Karyotype analysis and DEB/MMC test were requested for suspected FA. Result of the karyotype analysis was reported as normal but DEB/MMC test was consistent with FA. Requiring continuous transfusions, the patient was diagnosed with transfusion-dependent FA and was referred to another healthcare facility for bone marrow transplantation. Discussion – Conclusion: VACTERL association was found in our patient due to presence of anal atresia, tracheoesophageal fistula, renal agenesis and extremity defects. Thirty-three similar cases were found in the literature. Our patient presented to our clinic with pancytopenia when she was 8 years old. 24% of the cases reported had also hematologic complications during the first decade. In the literature review who had FA diagnosis with VACTERL association has 33% hypo-hyper-pigmentation, 76% renal abnormalities, 55% microphthalmiai/ dysmorphism, 24% genital abnormalities, 67% growth/developmental retardation,100% extremity abnormalities, 70% anal/duedenal atresia, 52% cardiac defect, %49 tracheoesophageal fistula/atresia, %30 hydrocephallus which our patient also had. In conclusion, we believe that it would be appropriate to evaluate patients who had FA diagnosis about VACTERL association. - 93 - Keywords: fanconi aplastic anemia, VACTERL association [P-184] Experiences on Neonatal Care After Fetal Surgery: First Cases of Turkey Nursu Kara1, Huseyin Canaz2, Ibrahim Alatas2, Kerem Ozel3, Ali Gedikbasi4 1 Department of Neonatology, Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey 2 Department of Neurosurgery, Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey 3 Department of Pediatric Surgery, Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey 4 Department of Perinatology, Kanuni Sultan Suleyman Training and Education Hospital, Istanbul, Turkey Myelomeningocele (MMC), one of the most common congenital malformations, can result in severe lifelong disabilities, including paraplegia, hydrocephalus, Chiari II malformation, incontinence, sexual dysfunction, skeletal deformations and mental impairment. MMC was the first nonlethal anomaly to be treated by fetal surgery. Fetal surgery was performed currently for several hundreds of human fetuses in North America and also for some fetuses in Brazil, Poland, Spain and Switzerland. Since preterm labor is one of the most common complications of fetal surgery, neonatologists must be aware of problems that can be faced off in these neonates. Fetal surgery requires a multidisciplinary team and neonatologist is one of the most important members. Our aim is to present the first two cases of Turkey and discuss the period in neonatal intensive care unit (NICU). Procedures were performed at respectively 25+2 and 25+4 weeks of gestation. Fetuses were delivered at 31+1 and 31+3 weeks of gestation and stayed 36 and 34 days respectively in NICU. Fetal surgery which is currently performed only in six centers in Europe, is a new procedure for our country. It is also a new point of interest and challenge for neonatologists. Keywords: Fetal surgery, myelomeningocele, neonatology [P-185] Jarcho Levine Syndrome: Experience on Neonatal Care Nursu Kara1, Huseyin Canaz2, Sukran Yildirim1, Banu Yazici3, Kerem Ozel4, Ibrahim Alatas2 1 Department of Neonatology, Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey 2 Department of Neurosurgery, Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey 3 Department of Pediatrics, Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey 4 Department of Pediatric Surgery, Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey Jarcho-Levin syndrome (JLS) is a segmental costovertebral malformation. It is a congenital disorder consisting of various vertebral and costal anomalies. Incidence is 0,25/10.000. Its main clinical signs are asymmetric chest wall, short neck-trunk stature scoliosis, and multiple radiological vertebral and related rib defects with mild thoracic restriction. It is composed of two subgroups: Spondylothorasic and spondylocostal dysostosis. Treatment requires earlier respiratory support and later orthopedic or surgical management. This syndrome is usually diagnosed in newborns. Spina bifida appears to be a common finding in reported JLS cases. The aim of this study is to report five cases with severe spondylocostal dysostosis and myelomeningocele and to discuss the complications in neonatal period. All the patients underwent surgery for myelomeningocele repair in the first 24 hours of life. Mean lenghth of stay in neonative intensive care unit was 16 days. Two patients died because of respiratory complications and one patient died due to cardiac complications. Congenital heart defects, abdominal wall malformations, urogenital and anal abnormalities, multiple skeletal anomalies, upper limb anomalies, spina bifida, and inguinal, umbilical and diaphragmatic hernias can be seen as components of JLS. Contents of JLS must be considered in neonates with myelomeningocele and spondylocostal dysostosis. Because JLS constitutes a challenge in neonatal care. Keywords: Jarcho Levine Syndrome, Myelomeningocele, Neonatal Care [P-186] A Newborn With Hemitruncus Arteriosus; Case Report Osen Ari1, Aras Tulunoglu1, Helen Bornaun2, Gökhan Büyükkale3, Abdullah Yahya Heybeci1, Murat Şahin2, Kazım Öztarhan2 1 Kanuni Sultan Suleyman Hospital, Pediatrics Department,,Istanbul, Turkey 2 Kanuni Sultan Suleyman Hospital, Pediatrics Cardiology, Istanbul, Turkey 3 Kanuni Sultan Suleyman Hospital, Neonatalogy, Istanbul, Turkey Introductıon: Wilson described the term Hemitruncus Arteriosus in 1798 as a congenital heart disease characterized by a single great artery that leaves the base of the heart giving rise to the coronary, pulmonary and systemic arteries. This term has fallen out of use, but it refers to a rare anomaly in which one pulmoner artery branch, usually the right, arises from the ascending aorta just above the aortic sinuses while the main pulmoner artery and the other pulmoner artery branch arise in their normal positions. Case Description: A 2-day-old male neonate delivered at term was referred to us due to poor feeding. He was tachypneic and tachycardic, his physical examination revealed no cardiac murmur. Chest X-ray showed cardiomegaly (cardiothoracic index was 0.60) with increased pulmonary arterial circulation. ECG was normal and right axis was dominant. Transthoracic Echocardiography revealed right pulmonary artery was arising from aorta approximately 1 cm from aortic valve leaflet and main pulmonary artery was continuous with the left pulmonary artery. Bifurcation was absent. Echocardiography also showed 7 mm ASD secundum. Discussion: In patient with anomalous origin of one pulmonary artery from the aorta, the affected lung is perfused at systemic pressure and remaining lung is - 94 - exposed to the entire output of the right ventricle. As the pulmonary resistance decreases in the neonatal period, the blood pressure and flow increases in the anomalous pulmonary artery causing pulmonary over circulation and pulmonary hypertension. As the mortality is very high in the first year of life early repair is mandatory to prevent development of irreversible pulmonary vascular disease. Conclusion: Hemitruncus arteriosus is an uncommon congenital cardiac malformation accounting for approximately %0.7 of all complex congenital heart lesions. Early diagnosis and pre- operative planning with management of pulmonary hypertension are necessary for successful outcome. Keywords: hemitruncus, congenital heart disease, truncus arteriosus drug on RBC surface and auto-antibody production resulting in IgG and/orIgM (C3d comploment) positivity in direct antiglobulin test. The most common causes of drug induced HA are penicillin and cephalosporins, non-steroidal anti-inflammatory and anti-neoplastics. In reported cases ceftriaxone is the most common drug which causes drug- iduced hemolytic anemia. Conclusion: Ceftriaxone- induced hemolytic anemia is rare but potentially fatal condition. In sick children who hospitalized for ceftriaxone treatment, hematocrit values and direct coombs testing must be evaluated closely for early diagnosis of drug-induced hemolytic anemia. Keywords: drug-induced hemolytic anemia, ceftriaxone [P-188] Guillian Barre Syndrome with CMV IgM Positivity [P-187] Ceftriaxone-Induced Immune Hemolyitic Anemia; Case Report Osen Ari1, Çağatay Günay1, Kenan Toprak1, Gökçen Kamış1, Özlem Başoğlu Oner1, Gönül Aydoğan3, Rengin Şiraneci2 1 Kanuni Sultan Suleyman Hospital, Pediatrics, İstanbul, Turkey 2 Kanuni Sultan Suleyman Hospital, Pediatric Infectious Disease Department, İstanbul, Turkey 3 Kanuni Sultan Suleyman Hospital, Pediatric Hematology and Oncology İstanbul, Turkey Introduction: Drug-induced hemolytic anemia (HA) is very rare state in childhood. Second and third generation of cephalosporins are the most common cause of drug induced HA. Case Description: A nine year–old boy with down syndrome was treated with ceftriaxone (100 mg/kg/ day) for acute gastroenteritis and pneumonia. While receiving ceftriaxone treatment, patient had developed anemia signs and symptoms. Hemolytic transfusion reaction to ceftriaxone was suspected because of the marked dropped in hematocrit value in the abscence of any overt clnical bleeding. The patient’s hematocrit value was 14% when patient deterirorated. Laboratory studies showed decrease in hemoglobin values from 14 gr/dl to 5 gr/dl, increase in LDH values to 476IU/L and (++++) direct coombs testing. Further evaluation revealed hemolysis on peripheral blood smear which demonstrated spherocytosis and polichromasia. He was treated with intravenous methyil-prednisolone 2mg/kg/ day, IVIG 1gr/kg for once, transfused with erytrocyte suspension, ceftriaxone was discontinued and antibiotic therapy was switch to vancomycin and clindamycin intravenously. The patient was discharged on the 30th day of the hospitalization with oral methtyl-prednisolone therapy, hematocrit value was 36%, direct coombs was evaluated (+) and C-reactive protein found negative. Discussion: There are three widely recognized mechanisms proposed to explain drug induced positive direct antiglobulin tests (diect coombs testing) and immune hemolytic anemia. Drug adsorption mechanism (hapten induced), immuncomplex formation with the Ozge Gucuyeter1, Tamay Gürbüz2, Behçet Şimşek3, Narin Akıcı4, Çiğdem Sağ5, Çağatay Nuhoğlu6 1 Özge Gücüyeter 2 Tamay Gürbüz 3 Behçet Şimşek 4 Narin Akıcı 5 Çiğdem Sağ 6 Çağatay Nuhoğlu Guillian-Barre Syndrome (GBS) is a postinfectious polyneuropathy involving mainly motor nerves. The initial diagnosis is based upon the clinical presentation and confirmed with albuminocytologic dissociation in cerebrospinal fluid and acute motor axonal polyneuropathy on electromyography. Plasmapheresis and intravenous immunoglobuline are the main treatment modalities. We present a case of Guillian-Barre Syndrome treated with both IVIG and plasmapheresis. A 16 year old female patient admitted with pain and weakness of legs and gait difficulty. On physical examination generalized muscle weakness of lower extremities were noted. In her blood tests CMV IgM was positive. Lumbar puncture was performed and albuminocytologic dissociation was detected. Acute motor axonal polyneuropathy in lower extremities was demonstrated on EMG and Guillian-Barre syndrome was diagnosed. IVIG was administered 1 gr/kg for two days. On the follow up the patient had progressive muscle weakness in spite of IVIG therapy and plasmapheresis was performed. Following seven sessions of plasmapheresis the patient markedly recovered from muscle weakness and was discharged on her twenty fourth day of hospitalisation. Data from the available small open-label randomized trials in children suggest that IVIG shortens the time to recovery compared with supportive care alone. In a meta-analysis of six trials and 649 patients with GBS that included a small number of children who were all >=10 years of age, treatment with plasma exchange was superior to supportive care. In our case, the patient had progressive muscle weakness and pain despite IVIG therapy. We performed plasma exchange on the fourteenth day of onset symptoms. After seven sessions of plasma exchange, the patient had remarkably less muscle weakness and pain. In conclusion, in the cases of Guillian-Barre Syndrome which are nonresponsive to - 95 - intravenous immunoglobulin therapy with progressive muscle weakness, plasmapheresis can be performed as a superior alternative choice of therapy. Keywords: guillianbarre, plasmapheresis [P-189] Three cases of anorexia nervosa with different presentations Övgü Kul Çınar1, Çiğdem Oruç1, Yağmur Birsev1, Oya Ercan2, Müjgan Alikaşifoğlu3 1 İ.Ü Cerrahpasa Medical Faculty Department of Pediatrics, Istanbul, Turkey 2 İ.Ü Cerrahpasa Medical Faculty Department of Pediatric Endocrinology, Istanbul, Turkey 3 İ.Ü Cerrahpasa Medical Faculty Department of Pediatrics, Adolescent Health Care, Istanbul, Turkey Background: Anorexia nervosa (AN) is a serious eating disorder characterized by abnormal eating behaviour, an intense fear of gaining weight, a distorted body image and psychiatric comorbidities. Anorexia nervosa often begins during the pre-teen or teen years or young adulthood and it is estimated to occur ten times more common in females. Cases: We present here three cases of AN who had long-term follow up in our adolescent health care clinic. All of them were females and they were; case 1:17 years 5 months, case 2:15 years, case 3:17 years 6 months). Only one of the patients admitted to hospital with a complaint of being extremely preoccupied with her appearance. The two other patients’ complaints were completely different at their admission. One of them admitted with constipation and the other with bone pain affecting both lower limbs. All three of the patients had secondary amenorrhea (7 months, 6 months, 6 months respectively), gastrointestinal symptoms as constipation and dyspepsia. While taking psychosocial history, we noticed that all three patients had distorted body image, they were on diet and perception of their weight had powerful influence on their self-evaluation, so we considered AN for diagnosis and consultated with psychiatry clinic. Their body mass indexes which are important to show the severity of disease were under 5 percentile. All the patients had vitamin B12, vitamin D and folate deficiency. One of the patients had decreased bone mineral density (Z score: -2,6). Results: For early diagnosis of AN, inquiring the body perception and eating behaviours must be a part of the routine evaluation of adolescents especially the ones whose BMI is under 5 percentile, who is on a diet or use other methods to lose weight, who has weight loss more than ten percentile of their body weight. Keywords: Anorexia nervosa, Distorted body image, Psychosocial history [P-190] Coexistence of immune thrombocytopenic purpura with cobalamin deficiency Özden Aksu Sayman, Zehra Esra Önal, Burcu Karadaş, Dilşad Koca Haydarpasa numune education and research hospital,istanbul,turkey Immune thrombocytpenia (ITP) of childhood is characterized by isolated immune-mediated thrombocytopenia. (peripheral blood platelet count<100,000/microl).It is an acqired and most commonly benign disorder.Nutritional megaloblastic anemias were chacterized over one hundred years ago with macroovalocytic red cells.The megaloblasts are products of impared DNA formation,due to deficiencies of cobalamin or folate. One year-aged male child was admitted to our clinic with the symptoms of petechiae,purpura and ecchymosis on the trunk.His physical examination was normal except hepatosplenomegaly.Laboratory studies; hemoglobulın:8.24g/dl, mean corpuscular volüme:82,5fl platelet count: 28x103 /µl cobalamin: 83pg/ml.The other whole blood examination were within normal limits.Examination of peripheral blood smear revealed normochromic,macrocyctic erythrocyte morphology, hypersegmented neutrophils and single-large platelets without clustered. We couldn’t examine bone marrow aspiration (no family consent). Thrombocytopenia related to immun thrombocytopenic purpura responsed to the treatment of intravenosus gamaglobulin,since cobalamin levels was lower than normal.So related with megaloblastic anemia, coexisted with ITP.We presented this case because of this coincidental presence of these two disorders. Keywords: cobalamin deficiency, ITP, megaloblastic anemia [P-191] Pulse oximetry screening for critical congenital heart disease in healty newborns before discharge Hacer Ergin, Özmert Muhammed Ali Özdemir, Özgün Taban, Ceren Çıralı, Merve Korkut Pamukkale University, Faculty of Medicine, Department of Pediatrics, Division of Neonatology, Denizli, Turkey Background and Aim: Congenital heart disease (CHD) occurs in approximately 8 out of 1000 live births. About 1-1.8 babies per 1000 live births have a duct dependent circulation, with a persistent ductus arteriosus being necessary for survival. A sizeable proportion of critical CHD (CCHD) is not detected by routine neonatal examination prior to discharge. Standard and portable echocardiograms are effective in identifying CCHD but are too costly to be used as a routine screening method. The aim of this study is to evaluate the use of pulse oximetry for early detection of CCHD. Methods: This study included neonates born in Pamukkale University Hospital and followed in well baby nurseries. Newborns, prenatally diagnosed with duct dependent circulation and admitted to neonatal intensive care unit, were excluded. Oxygen saturation - 96 - screening was conducted preductally (palm of right hand) and postductally (either foot) with identical pulse oximetry. When both preductal and postductal oxygen saturations were <95% or the difference between the two measurements was >3%, the newborn was considered to be screening positive. Newborns with abnormal cardiac findings such as murmurs, tachypnea, overt cyanosis, and had three repeated positive measurements were supposed to have an echocardiogram. Results: Of the 859 newborns eligible for the screening study, 657 (76.4%) had complete data from both pulse oximetry and physical examination. Of the 657 infants with complete screening data, 8 (1.2%) had positive pulse oximetry results, and 65 (9.8%) had abnormal cardiac findings. While diagnostic echocardiogram showed patent foramen ovale in 6 infants whose pulse oximetry results were positive, none had ductus dependent CCHD. Conclusion: Pulse oximetry screening is an effective, noninvasive, and inexpensive tool allowing earlier diagnosis of CCHD. Newborns with positive screen should evaluate for diagnostic echocardiogram and pediatric cardiology consultation. Keywords: pulse oximetry, congenital heart disease, newborn [P-192] An infant with Herpes Simplex Encephalitis: A case report Özlem Öçal Doğan1, Nihat Çelebi1, Pınar Yamaç Dilaver1, Ece Gültekin2, Leyla Telhan3, Nalan Karabayır4, Güzide Baysan Turanlı2 1 Medipol Univercity, Department of Pediatrics 2 Medipol Univercity, Department of Pediatric Neurology 3 Medipol Univercity, Department of Pediatric Intensive Care Unit 4 Medipol Univercity, Department of Social Pediatrics Background&Aims: Herpes simplex encephalitis (HSE) is the most common cause of viral encephalitis in infants and children and it is a devastating disease that can be difficult to diagnose in its early stages. Treated or untreated, it can be associated with considerable morbidity and mortality. In this report, we present a case of herpes simplex encephalitis, 4,5 month-old male patient, who admitted to our emegency department with fever, diarrhea and focal seizures. He had bulging fontanelle, focal tonicclonic seizures on the left arm, no eye contact. Other physical examinations, blood count, biochemical and stool tests were all normal. HSV was subsequently detected by PCR in the cerebrospinal fluid (CSF). MRI scan demonstrated features consistent with meningoencephalitis on temporal lobe involvement. Periodic lateralising epileptiform discharges in the right temporal lobe and abnormal background activity on the right hemisphere were EEG findings. High-dose intravenous(IV) ceftriaksone and acyclovir were initiated immediately. The antiepileptic treatment decreased the frequency of seizures. The patient who completed 7 days of ceftriaksone and 21 days of antiviral therapy were discharged on day 32. The patient is periodically checking by neurology and psychological and educational rehabilitation. Conclusions: In conclusion, herpes virus infections are often asymptomatic in the infancy period. An infant who presents with fever, altered mental state (encephalopathy) and focal seizures or focal neurological abnormalities, we should also consider HSV encephalitis. Earlier antiviral therapy is associated with a better outcome, which is very important in reducing morbidity and also mortality. Keywords: fever, seizure, herpes simplex encephalitis [P-193] Orbital cellulitis in a forty-five day old baby Yelda Türkmeoğlu1, Elif Arslan1, Pınar Bütün1, Sibel Aşkın Perkin1, Vefik Arıca2, Gülşen Köse3 1 İstanbul Okmeydanı Eğitim ve Araştırma Hastanesi Çocuk Sağlığı ve Hastalıkları 2 İstanbul Yeni Yüzyıl Üniversitesi Tıp Fakültesi Gaziosmanpaşa Hastanesi Çocuk Sağlığı ve Hastalıkları 3 İstanbul Şişli Hamidiye Etfal Eğitim ve Araştırma Hastanesi Çocuk Sağlığı ve Hastalıkları Preseptal cellulitis is a mild infection of the structures in front of orbital septum characterized by the edema, tenderness and erythema of the eyelid. Whereas orbital cellulitis is the infection of adnexial tissues composed of the fat and muscle, behind the orbital septum, in addition to the signs of preseptal cellulitis, there is proptosis and limitation of ocular movements. In addition to loss of vision, it has life- threating complications, such as intracranial infections and cavernous sinus thrombosis. For this reasons its urgent differential diagnosis from preseptal cellulitis and treatment are crucial. Although orbital cellulitis is more common in children than in adults, it is very rare in babies younger than six - month. In this article a case of orbital cellulitis, whom was treated with appropriate antibiotic therapy without any complication, in a 45-day old baby is reported. Keywords: infant, newborn, orbital cellulitis [P-194] Successful Hyperbaric Oxygen Treatment of Bilateral Refractory Gangrenous Lesions due to Systemic Lupus Erythematosus Pınar Özge Avar Aydın1, Çiğdem Aktuğlu Zeybek1, Ertuğrul Kıykım1, Akın Savaş Toklu2, Ozgur Kasapçopur3 1 Department of Pediatric Nutrition and Metabolism, Istanbul University Cerrahpasa Faculty of Medicine, Istanbul, Turkey 2 Department of Underwater and Hyperbaric Medicine, Istanbul University Faculty of Medicine, Istanbul, Turkey 3 Department of Pediatric Rheumatology, Istanbul University Cerrahpasa Faculty of Medicine, Istanbul, Turkey Background&Aims: Necrotizing vasculopathy is a relatively uncommon and life-threatening condition in children. Raynaud phenomenon and digital ischemia are the most common symptoms. Although immunosuppressive treatments in combination with vasodilators are the first chosen therapy methods hyperbaric oxygen therapy is also used as primary or - 97 - [P-196] adjuvant therapy in some. Case: We report a case of 15 years-old female patient who presented with gangrenous lesions at her feet phalanges and hyperglycemia had been diagnosed as diabetes mellitus type 1 and systemic necrotizing vasculitis due to systemic lupus erythematosus. Effective treatment by hyperbaric oxygen therapy in combination with immunosuppressive and vasodilator drugs with supporting therapies led to significant improvement in gangrenous lesions without amputation. Conclusion: Adjunctive hyperbaric oxygen therapy should be considered in cases with progressive gangrenous lesions due to vasculopathies. Keywords: Hyperbaric oxygen treatment, systemic necrotizing vasculitis, systemic lupus erythematosus [P-195] Different Clinical Spectrum of Leptospirosis Fatma Deniz Aygün, Pınar Özge Avar Aydın, Haluk Çokuğraş, Yıldız Camcıoğlu Division of Pediatric Infectious Diseases, Clinical Immunology and Allergy, Istanbul University Cerrahpasa Faculty of Medicine Background&Aims: Leptospirosis is a prevalent zoonotic disease. Human infection usually occurs through exposure to environmental sources. Clinical course of leptospirosis is variable. Cases: We presented five patients, aged between 4-14, having a history of contact with rodents and symptoms 7-10 days after contact. The first three cases were relatives and had contact with dead rats after applying insecticides to bakery opposite to their house. The first case diagnosed as isolated meningitis, others as flulike illness. The fourth case had a contact history with a rat inundate in the canalization and diagnosed as acute hepatitis. The last case, living in a village with poor sanitation, developed secondary hemophagocytic syndrome. ELISA was performed for diagnosis. High dose penicillin and additional immunsuppressive drugs for the last case were used. All cases showed recovery within 10 days. Conclusion: Leptospirosis should be considered in any patient presenting with an abrupt onset or prolonged fever, myalgia, headache and jaundice. Keywords: leptospirosis, meningitis, hemophagocytic syndrome Peripherally Inserted Intravenous Catheters in Children: One Year Survelliance Fatih Aygün1, Fatma Deniz Aygün2, Pınar Özge Avar Aydın1, Halit Çam1 1 Istanbul University, Cerrahpasa Medical Faculty, Pediatric Intensive Care Unit, Istanbul, Turkey 2 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatric Infectious Diseases, Istanbul, Turkey Aims: Intravascular catheters are used with increasing frequencies in the management of children. The aim of this study is to evaluate the complications and risk factors associated with intravascular catheterization. Methods: Between the years 2014-2015 Augusts, in Medical Faculty of Cerrahpasa, University of Istanbul, Pediatric Intensive Care Unit, 105 patients and placed 171 intravascular catheters analyzed retrospectively by the patients’ medical records. Results: Average age of catheter insertion was 4,45±5,48 years, ranging between 3 days-228 months. 78 of the patients (45.6%) were female, 93 (54.4%) were male. One hundred and fifteen catheters (67.3%) were used 7 days or more, the average length of usage was 20.33±38.80. The most common underlying disease was metabolic disease (n = 43, 25.1%). Catheters were inserted most often because of the absence of a peripheral venous route (n = 77, 45%). The most common catheter types were central venous catheter (n = 110, 64.3%) and hemodialysis catheters (n = 51, 29.8%), respectively. Ninety eight femoral (57.3%), 48 jugular (28.1%) and 22 subclavian (12.9%) catheters were placed. Hundred and seven of the catheters (62.6%) were used only in intensive care unit, 30 (17.5%) in services after intensive care unit and 34 catheters (19.9%) were placed for the usage in services. Catheter-related infections were observed in 13 patients (7.6%). Noninfectious complications were observed in 6 cases (3.5%) during or after insertion. Ninety five catheters (55.6%) withdrew due to no more remaining requirement. As catheter type, size and location had no risk for catheter related infections (p> 0.05), increased time length for the usage of catheters showed a significant increase in infection incidence (p <0.05). The frequency of infection of catheters followed in services was significantly higher (p <0.05). Conclusions: The insertion and care of catheters by skilled persons reduce complication rates. Keywords: Children, intravascular catheter, catheter complications - 98 - [P-197] [P-198] Two Cases with Systemic Trombosis as a Complication of Intraarterial Chemotherapy for Retinoblastoma Pınar Özge Avar Aydın1, Ahmet Sarıcı2, Osman Kızılkılıç3, Tiraje Celkan1 1 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatric Hematology-Oncology, Istanbul, Turkey 2 Istanbul University, Cerrahpasa Medical Faculty, Department of Ophthalmology, Istanbul, Turkey 3 Istanbul University, Cerrahpasa Medical Faculty, Department of Radiology, Istanbul, Turkey Background&Aims: Retinoblastoma (Rb) is the most common intraocular tumor in childhood. Conventional treatment methods include systemic chemotherapy, enucleation, external beam radiotherapy and focal therapies, such as cryotherapy, thermotherapy, laser photocoagulation. Intraarterial chemotherapy (IAC) for advanced and refractory Rb has become popular recently in an attempt to avoid enucleation and systemic complications. Common minor ocular side effects occur in many cases and can resolve spontaneously within a few months. Common systemic complications include neutropenia, bronchospasm and allergy. Local complications at femoral puncture side are possible. Systemic vascular complications are rare. Herein, we report two cases with Rb in which extraocular complications after IAC with melphalan. Cases:A 2,5 months old boy was diagnosed as bilateral Rb. The patient underwent local cryotherapy and systemic chemotherapy. Because of his refractory Rb in his left eye IAC with melphalan was performed. Two days after the first IAC, the patient was admitted with confusion and emesis. On his physical examination, there was right hemiplegia and hemiparesis. Cranial CT scan and MRI were performed and revealed acute ischemic lesion in the territory of the right middle cerebral artery. He was given parenteral low-molecular-weight heparin for 14 days. Hemiplegia didn’t resolve completely and he was not given second cycle of IAC. Second case is a 7 months old boy with unilateral Rb. He was given 3 cycles of IAC. After the third IAC, he was admitted with a painful, blue left toe. He was given 14 days of both oral aspirin and low-molecular-weight heparin due to blue toe syndrome. After 2 weeks, the apperance of left foot was totally normal. Conclusions: IAC is milestone of the treatment of refractory and advanced Rb in children. It may cause systemic vascular complications which are rare but serious. Patients may be analysed for tendency to trombosis and systemic vascular complications should be defined. Keywords: retinoblastoma, intraarterial chemotherapy, trombosis Juvenile Dermatomyositis: Clinical Features, Laboratory Findings, Treatment Modalities and Disease Course (A Single Center Experience From Turkey) Özgür Kasapçopur1, Pınar Özge Avar Aydın2, Kenan Barut1, Salim Çalışkan3, Lale Sever3, Nil Arısoy1 1 Department of Pediatric Rheumatology, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey 2 Department of Pediatrics, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey 3 Department of Pediatric Nephrology, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey Introduction: Juvenile dermatomyositis (JDM) is the most prevalent idiopathic inflammatory myopathy of childhood and characterized by proximal muscle weakness and typical skin involvement. Objectives: To describe demographics, clinical features, laboratory findings and treatment modalities of patients with juvenile dermatomyositis (JDM) at a referral pediatric rheumatology center in Turkey. Methods: Retrospective review of forty three patients meeting the Bohan and Peter criteria diagnosed as JDM at the Pediatric Rheumatology Department of Istanbul University Cerrahpasa Medical Faculty between the years 2003-2013. Results: Forty three patients were identified; 30 of them (69 %) were female. Mean age for the onset of the disease was 6.3, mean age for the diagnosis was 6.9 years. Mean follow-up period was 48 months. As the most common clinical presentations, heliotropic rash and Gottron papules were seen in 42 patients (97 %), muscle weakness was seen in 39 patients (90 %), erythroderma in 36 patients (87 %) and calcinosis in 16 patients (37 %). All patients had elevated muscle enzymes at the diagnosis. Twenty seven patients (62 %) had anti-nuclear antibody (ANA) positivity. Muscle biopsy was performed on 14 patients (32 %) and all of them showed histopathological changes of inflammatory myositis. Electromyography was performed on 25 patients (58 %) and all of them recorded myopathy. All patients had been treated with corticosteroids at different dosages. Methotrexate was used in 41 patients (95 %), cyclosporine was used in 16 patients (37 %). All patients except two cases, who were referred to our clinic after a long duration of the disease and had widespread calcinosis, achieved remission. Conclusion: Our patients with JDM have similar characteristic findings compared with previously described cohorts. Awareness about JDM diagnosis and treatment should be increased among pediatricians. Keywords: Juvenile dermatomyositis, muscle weakness, skin rashes - 99 - [P-199] [P-200] Pınar Özge Avar Aydın1, Bahar Özcabı2, Aydilek Dağdeviren Çakır2, Oya Ercan2, Olcay Evliyaoğlu2 1 Cerrahpasa Medical Faculty of Istanbul University, Department of Pediatrics, Istanbul, Turkey 2 Cerrahpasa Medical Faculty of Istanbul University, Department of Pediatric Endocrinology and Diabetes, Istanbul, Turkey Background&Aims: The evaluation of the patients diagnosed with growth hormone deficiency and treated with growth hormone for at least one year and the analysis of the factors affecting growth rates. Nalan Karabayir1, Dogukan Aktas2, Kazim Oztarhan3, Emre Kelesoglu1, Abdullah Erdem2, Helen Bornau3, Pinar Yamac Dilaver1 1 Pediatrics Department, Faculty of Medicine, Istanbul Medipol University, Istanbul, Turkey 2 Pediatric Cardiology Department, Faculty of Medicine, Istanbul Medipol University, Istanbul, Turkey 3 Pediatric Cardiology Department, Kanuni Sultan Suleyman Training and Education Hospital, Istanbul, Turkey Dilated cardiomyopathy, which mostly has an idiopathic etiology or is caused by genetic inheritance or infection, may cause irreversible congestive heart failure. Hypocalcemia is a rare etiology of reversible dilated cardiomyopathy. Cardiogenic shock related to hypocalcemic cardiomyopathy is a rare event. Here we report two cases with congestive heart failure who was diagnosed as having dilated cardiomyopathy secondary to hypocalcemia. After calcium and vitamin D replacement therapy, the patients showed improvement in left ventricular function. Hypocalcemia should be included in the differential diagnosis of dilated cardiomyopathy. Keywords: cardiogenic shock, dilated cardiomyopathy, vitamin D deficiency Growth Hormone Therapy in Childhood: A Single Center Experience Methods: Patients diagnosed with growth hormone deficiency at the Pediatric Endocrinology Department of Cerrahpasa Medical Faculty of Istanbul University between the years 2008-2014 and treated with growth hormone for at least one year were analyzed retrospectively. Results: One hundred and eight patients were identified; 55 of them (50,9%) were female. Mean age at the admission was 9,72±3,50. Six patients were treated with GH diagnosed with multiple hypophyseal hormone deficiency, 9 patients with Turner Syndrome, 6 patients with rheumatic diseases and 11 patients with other diagnoses. Mean responses were 3,63±2,31 ng/ml on levodopa and 4,91±2,58 ng/ml on clonidine tests. Forty six patients (42,6%) were diagnosed as complete and 62 (57,4%) as partial GHD. Sella MRI was obtained normal in 81,5% of the patients. Thirty one of the patients (28,7%) were pubertal. The most commonly seen side effects were as follows: hyperglycemia (14%), elevated muscle enzymes (10,3%) and hyperinsulinemia (10,3%). The highest height velocity was detected during the first year (8,29±2,55 cm) and decreased gradually during following years. Mean delta boy SDS was 0,90±0,89. Genetic consistency was medium (-1,62±1,50) before GH therapy while good (-0,68±1,16) after GH therapy, respectively. Conclusions: Growth hormone therapy is safely used in children with increasing frequency. The highest height velocity was detected at the first year of the therapy. There are several factors affecting growth velocity and the effectiveness of the therapy may show individual differences. In this study, the most important factors affecting growth response were age and height SDS before the treatment. The importance of early diagnosis and treatment were desired to be emphasized with these results. Keywords: Growth hormone, growth hormone deficiency, children Dilated Cardiomyopathy due to Vitamin D Deficiency [P-201] Bilateral Uretheral Stones Mimicking Hemolytic Uremic Syndrome Cihangir Akgun1, Ayse Serap Akgun2, Mehmet Gunduz3, Pinar Yamac Dilaver3, Vahit Guzelburc4, Meliha Aksoy3, Mesut Okur3 1 Pediatric Nephrology Department, Faculty of Medicine, Istanbul Medipol University, Istanbul, Turkey 2 Radiology Department, Faculty of Medicine, Istanbul Medipol University, Istanbul, Turkey 3 Pediatrics Department, Faculty of Medicine, Istanbul Medipol University, Istanbul, Turkey 4 Urology Department, Faculty of Medicine, Istanbul Medipol University, Istanbul, Turkey A four years old boy was admitted to our hospital with total anuria and facial swelling which started one day ago. Except mild facial edema, he had no pathologic findings on his physical examination including costovertebral angle tenderness. Bilateral grade 1 renal parenchymal disease, grade 2 hydronephrosis have been reported and no stone has been identified on his ultrasonographic evaluation of urinary tract. Initially, the patient was diagnosed as hemolytic uremic syndrome due to the history of bloody diarrhea, acute renal failure, thrombocytopenia and anemia so hemodialysis was performed immediately, by the way laboratory examination was continued. There was no sign for microangiopathic hemolytic anemia at peripheral blood smear and other laboratory values were normal. Then, for the possibility of obstructive pathology computed tomography was performed and bilateral uretheral stones were seen. After double j stent have been placed to both renal pelvis due to fluoroscopy by urologist, urine output was normalized and the patient - 100 - has been discharged. Bilateral uretheral obstruction due to stone is a very rare condition in children, underlying reasons have to identify. Non-contrast computed tomography can be helpful especially in non-opaque stone. Keywords: bilateral renal stone, anuria, hemolytic uremic syndrome [P-202] Nutcracker Syndrome in Siblings Without Hematuria Cihangir Akgun1, Ayse Serap Akgun2, Mehmet Gunduz3, Gizem Ondalikoglu3, Pinar Yamac Dilaver3, Mesut Okur3, Arda Ozyuksel4 1 Pediatric Nephrology Department, Faculty of Medicine, Istanbul Medipol University, Istanbul, Turkey 2 Radiology Department, Faculty of Medicine, Istanbul Medipol University, Istanbul, Turkey 3 Pediatrics Department, Faculty of Medicine, Istanbul Medipol University, Istanbul, Turkey 4 Department of Cardiovascular Surgery, Faculty of Medicine, Istanbul Medipol University, Istanbul, Turkey Introduction: Nutcracker syndrome (NCS) refers to compression of LRV (left renal vein) between aorta and superior mesenteric artery (SMA). The patients have symptoms such as hematuria, proteinuria, and left flank pain. The following frequency of symptoms was noted: 78.57% for haematuria, 38.39% for left flank pain, 35.71% for varicocele in males, 30.36% for proteinuria and 13.39% for anaemia. Isolated hematuria was reported 33.3% in children with NCS. Microhematuria is 4 times more common than macrohematuria.Orthostatic proteinuria is another common symptom in NCS. Here, we present two new cases of NCS in a young male person and his sister. Case 1: 15 years old female patient was admitted to our outpatient clinic with proteinuria that detected while swimming pool registery tests. Only proteinuria was found on urinalysis. In the history of the patient, kidney biopsy was performed for proteinuria before refferred to our hospital and the result was normal. MR of the abdomen was requested for further investigation of proteinuria. A diagnosis of nutcracker syndrome was established. Case 2: 18 year-old male patient whose sister was diagnosed as NCS. His blood tests were normal and only proteinuria was found on urinalysis. Bilateral renal artery MR performed. MR eventually revealed pressed left renal vein due to compression between the superior mesenteric artery (SMA) and the abdominal aorta, consistent with NCS. The patient had also left varicocele. Discussion: Eventough, NCS is frequently associated with hematuria,it may present with only proteinuria without hematuria. NCS must be considered in different diagnosis of isoleted proteinuria. We report two cases of family members with NCS. It is important in terms of being the first case that seen in siblings, according to our knowledge. Keywords: nutcracker, siblings, wıthout hematuria [P-203] Seizure and loss of consciousness in an adolescent: mitochondrial disease Püren Özüdoğru1, Özlem Sert1, Nilgün Erkek2 1 Akdeniz University Faculty of Medicine, Department of Pediatrics, Antalya 2 Akdeniz University Faculty of Medicine, Division of Pediatric Emergency, Antalya Background&Aims: A seizure represents the clinical expression of abnormal, excessive, synchronous discharges of neurons, which makes transient changes in behavior, movement and sensation for a short time. The etiology of seizures is multifactorial, one of these is thromboembolism. The aim of this case report is to indicate that mitochondrial diseases can lead to thromboembolic episodes, which can come up with seizures and changes in consciousness. Methods: A 17 year old boy with seizure and acute loss of consciousness hospitalized in the department of pediatrics. His glasgow coma scale was 8 and laboratory investigations were normal. The neurological status came to normal at the 12th hour of treatment, except for the articulation deficit, which he had also before. His cranial MR-screening showed restricted diffusion at the right parietooccipitotemporal area, indicating thrombembolism. His genetic screening for thrombophilia was MTHFR 677- heterozygote mutant. MR spectroscopy showed lactate changes at the area of thromboembolism, suggesting MELAS. We performed genetic analysis for mitochondrial diseases, in which %95 of the responsible genes were studied and none of them were mutant. Results: There are multiple risk factors for thromboembolism in children (anatomic, genetic and acquired). In our case we found it interesting that the patient had MTHFR 677 gene heterozygote mutant and he had lactate changes in MR spect. Conclusions: This report highlights the role of mitochondrial diseases in the etiology of thromboembolism and seizure Keywords: seizure, thromboembolism, MELAS [P-204] A severe case of neonatal hypotonia Raffaella Panza1, Annalisa Resta1, Alessandro Nicola Abbaticchio1, Donatella Capodiferro1, Nicoletta Resta2, Lucrezia De Cosmo1, Nicola Laforgia1 1 Department of Neonatology and NICU, University of Bari, Italy 2 Department of Human and Medical Genetics, University of Bari, Italy “Floppy infant” is an hypotonic newborn who may present respiratory distress at birth, low Apgar score, weak sucking and swallowing. The diagnosis is difficult in the neonatal period as it may be related to numerous conditions. An accurate neurological examination, the assessment of Precthl’s General Movements, the study of spontaneous motility of passive and active muscle - 101 - tone and primitive reflexes, may help understand if hypotonia is due to central or peripheral causes. MRI, US, laboratory tests, specific tests such as muscle biopsy, EMG, genetic tests are usually required. A newborn, born at 42+4 weeks of GA by vaginal delivery, was admitted to our UTIN because of perinatal asphyxia and required therapeutic hypothermia and ventilatory support for one month; the patient died on the 30th DOL. She presented severe generalized hypotonia, no spontaneous movements of the limbs, inappropriate suck-swallow pattern, no deep tendon reflex. The mother reported reduced fetal movements and polyhydramnios. Brain MRI was normal, Total Body Rx revealed multiple spontaneous fractures of tibia and femur, EMG showed absence of spontaneous and induced motility, and muscle biopsy showed non-specific signs of myopathy. Karyotype and genetic test for Prader-Willi were normal. Molecular test for Spinal Muscular Atrophy (SMA) revealed a non-funcional point mutation of the SMN1 gene and only one copy of the SMN2 gene. SMA I with neonatal onset, named SMA 0, always carries a fatal prognosis. It is usually due to an omozygotic mutation of the SMN1 gene. Moreover, copy-number variation of the SMN2 gene seems to be linked to the disease onset: the lower the number of copies (e.g. 1 or 2) the worst both clinical presentation and prognosis. The association of a SMN1 gene non-functional mutation with a single copy of the SMN2 gene could explain the clinical severity and negative rapid outcome, similar to SMA 0. Keywords: floppy infant, hypotonia, SMA [P-205] Meningitis with a history of head trauma: a case report Emine Olcay Yasa, Refia Gözdenur Savcı, Müferet Ergüven İstanbul Medeniyet University Göztepe Training and Research Hospital, Pediatrics Department, İstanbul, Turkey Introduction: Meningitis is an inflamatory disease of pie and arachnoid membranes surrounding the brain and spinal cord. Meningitis is a medical emergancy and the possibility of meningitis increases after head injury and skull fractures. This may be recurrent and causing agent is often S. pneumoniae. Case: A 8 year old previosly healthy female child presented with the complaints of fever, headache, drowsiness, diplopia and altered consciousness.Her mother told that she had stared at ceilling and hadn’t responded to any stimulus. She was feverish and had a poor general appearance. Neurologic examination revealed nuchal rigidity. Because of her ongoing confusion we had a cranial CT scan. It showed us the asymetry of lateral venticles. In laboratory findings, WBC:33500/ mm3, %84 NEU, Hb:13,9 gr/dl, Hct:43%, PLT:251000/ mm3, CRP:7,8 mg/dl.There was contrast enhancement in leptomeningeal area in cranial MRI. There was no papilledema in fundus examination. We performed a lumbar puncture and found that WBC: 12/mm3 ( %80 Lymph), Erythrocyte: 2/mm3, Glu: 53 gr/dl (SBG: 80 gr/dl), protein 47 mg/dl, Na: 140 mEq/lt. We started Ceftriaxon for empirically and mannitol due to increased intracranial pressure. In detailed history of the patient we learned that she had fallen from the first floor and had got an epidural hematoma and a skull fracture two years ago. Therefore we added vancomycine to avoid s. pneumoniae. The patient’s fever dropped, neurological symptoms were improved, her antibiotics were completed and she was discharged. In follow up visit we found that she had a CSF fistula in MR cisternography. We directed her to brain surgery department to eveluate for operation. Conclusion: Meningitis is the one of the most common infectious disease of childhood. A detailed history would help to eluciate the underlying cause and to avoid recurrency. Keywords: head trauma, skull fracture, meningitis [P-206] Perforated appendicitis: is it a challenging diagnosis in children? Gülser Esen Besli1, Refia Gözdenur Savcı1, Müferet Ergüven1, Çiğdem Ulukaya Durakbaşa2 1 İstanbul Medeniyet University Göztepe Training and Research Hospital, Pediatrics Department, İstanbul, Turkey 2 İstanbul Medeniyet University Göztepe Training and Reseach Hospital, Pediatric Surgery Department, İstanbul, Turkey Introduction: Acute appendicitis is the most common non-traumatic emergency surgery in childhood. Its mortality and morbidity are very low when a prompt diagnosis and an early treatment are carried out. Delayed diagnosis can cause severe complications. This report highlights two cases of perforated appendicitis with severe clinical presentations having malignancy suspicion initially. Case 1: A 16 year old male presented with a three week history of abdominal pain and weight loss. He was referred from another institution with diagnosis of abdominal tumour. He looked unwell, apathetic, and had fever and a tender pelvic mass. An abdominal ultrasonography reported a solid mass lesion located behind the bladder. An abdominal CT showed an abcess which started supravesical pelvic region and extanding rectovesical recess. He was operated on for perforated appendicitis and a large pelvic appendiceal abscess was drained. Case 2: A ten year old girl had admitted to a hospital with a four week history of nausea, vomitting, abdominal pain, and respiratory distress. She referred to our hospital with a suspicion of malignancy due to pleural effusion, ascites, hepatomegaly and weight loss. She looked unwell, cachectic, and had tachypnea, dyspnea, and distended abdomen. His breath sounds were decreased especially in the right hemithorax. She had diffuse abdominal tenderness. An abdominal CT showed located collections in perihepatic and perisplenic areas and a fecalith in the appendix. There were bilateral atelectasis and pleural effusion in the right lung on thorax CT. She was operated on for perforated appendicitis, abdominal absess and generalized peritonitis. - 102 - Discussion: Delayed diagnosis of acute appendicitis can cause life-threatening conditions such as abdominal abscess and sepsis. Extraabdominal complications like pleural effusion and empyema may occur. It should also be considered that childhood appendicitis may mimic pelvic malignancy. We emphasize the importance of early treatment for acute appendicitis and septic complications. Keywords: perforated appendicitis, abdominal absess, malignancy [P-207] One of the major causes of pleural effusions: Tuberculosis Emine Olcay Yasa, Refia Gözdenur Savcı, Müferet Ergüven, Şeyma Sönmez Şahin İstanbul Medeniyet University Göztepe Training and Research Hospital, Pediatrics Department, İstanbul, Turkey Introduction: Tuberculosis(TB) is an infectious disease caused by Mycobacterium tuberculosis Even if it is a preventable and curable disease TB remains as one of the most common causes of mortality and morbidity worldwide Case: A 13-year-old female child presented with the complaints of high grade fever, cought and back pain for the last 3 weeks. She had received several courses of antibiotics without any response. Thoracentesis had been performed to her grandmother because of pulmonary infection but she didn’t live in the same house. She had showed good general state of health. Height and weight were within limits compatible with the patient’s age. She was feverish. Respiratory system eveluation was suggestive of a right sided pleural effusion. A chest radiograph and thorasic ultrasonography confirmed the pleural effusion and atelectatic areas in the right hemithorax. CBC and biochemical analysis were normal but acute phase reactants were significantly higher. Rheumotologic markers were evaluated to exclude rhemotologic diseases. We started empirical antibiotic therapy. Thorasentesis was performed by interventional radiologists. In laboratory findings of samples; it was pale yellow, cell count was 3440 Leukocyte/mm3 ( %90 Lymphocyte), 3600 Erytrocyte/mm3 and biochemical analysis revealed that Glu: 93 mg/dl, Cl: 107 mEq/L, protein 5,4 gr/dl, ADA 40,9 U/L. Microbiological examination of fluid was normal. A Tuberculin skin test (TST) performed with a markedly positive result after 72 hours. All family members were screened in tuberculosis dispansaries. Because of lack of clinical benefit from antibiotic therapy in last 3 weeks, positive TST, characteristics of pleural fluid examination, and suspicious family history we diagnosed as pleural TB and started antiTB treatment including İsoniazid, Rifampin and Pyrazinamide. The patient’s fever dropped, her respiratory symptoms were improved and she was discharged. Periodic follow-up visits are ongoing. Conclusion: TB should be kept in mind in expecially feverish and antibiotic refractory cases. Keywords: tuberculosis, pleural effusions [P-208] Multiple avascular necrosis in a pediatric antiphospholipid syndrome secondary to systemic lupus erythematosus - clinical case Rodica Eremciuc, Prof. Ninel Revenco, Tatiana Macovei Pediatric Department, State University of Medicine and Pharmacy “Nicolae Testemitanu”, Chisinau, Republic of Moldova Background&Aims: Avascular necrosis (AVN) occurs in several chronic illnesses, including systemic lupus erythematosus (SLE). The mechanism about why and how AVN occurs isn`t unraveled yet. AVN in the pediatric lupus population is understudied. Methods: We report a case of a 15-year-old girl, who was on record in the Rheumatology Clinic over the last year with the diagnosis of SLE with multiorgan involvement associated with secondary antiphospholipid syndrome (immunologically confirmed by the presence of positive ANA, anti-dsDNA, antiphospholipid and anticardiolipin antibodies). The exacerbation of the articular syndrome 9 months after onset of disease required a bilateral CT scan of the coxofemoral and knee joints. Results: Clinical examination of the child 9 months after onset of disease showed the exacerbation of the inflammatory articular syndrome expressed by manifest, prolonged, treatment-resistant pain in the coxofemoral (R>L), bilateral knee and left talocrural joints (8090 mm along VAS). Swelling of the both knees and both talocrural joints was determined on the clinical assessment. On paraclinical examination persisted increased inflammation acute phase reactants. X-ray revealed erosions and signs for lacunar osteoporosis in the knees and coxofemoral joints. The CT-scan of the coxofemoral joint showed a marginal fracture of the right capitis femoris (the fragment size 16x28mm) with dislocation of 2mm. Knee CT-scan presented bilateral AVN of femoral condyles, with signs of pathologic fracture at these levels. Further therapeutic management of this case is being realized in a multidisciplinary team, that consists of pediatric rheumatologists and orthopeds. Conclusions: AVN can cause significant morbidity. No single etiology is known to cause the interruption of blood supply which is the common pathway for all AVN; rather, AVN is likely the end result of a multifactorial process. Considering the age of this patient, periodic follow-ups with an orthopedic specialist are essential for a good long-term outcome. Keywords: Avascular necrosis, Secondary antiphospholipid syndrome, Systemic lupus erythematosus [P-209] Mixed Germ Cell Tumor In Down Syndrome Ruhsar Erdoğmuş, Muhammed Nurullah Yakut, Süheyla Ocakçı, Hilal Şen İstanbul Okmeydanı Eğitim Araştırma Hastanesi Introductıon: Down syndrome (trisomy 21) is the most common and best known chromosomal disorder in - 103 - humans. Only a few cases of testicular germ cell tumor in Down Syndrome (DS) children have been reported in literature. We present here an adolescent boy with DS and testicular mixed germ cell tumor. Case: 15-years-old boy with DS referred to our outpatient clinic due to a left testicular mass. He has not been on regular follow up for DS. A painless lump on the left testis has been first noticed about one month ago. He had no history of criptorchidism. Physical examination revealed a 5x6 cm, painless, hard, well-defined left testicular mass. Right testis was normal. Serum AFP was 752 ng/ml (N < 7 ng/ml) and B-HCG was 275 miu/ml (N < 0,5 miu/ ml). Scrotal ultrasonography (USG) revelaed a 63x41 cm left testicular hypervascular mass with scattered microcalcifications. Abdominal USG and chest x-ray were normal. Left orchiectomy with high inguinal ligation was performed. Pathologic examination was reported as mixed germ cell tumor ( embriyonal carcinoma 50%, yolk sac tumor 30%, immature teratoma 20%), with negative surgical margins. Post-operative serum AFP, B-HCG levels, scrotal USG, abdominopelvic MRI and Thorax CT were all normal. He was considered as Stage I, testicular mixed germ cell tumor. He was planned to be on close follow up with no additional treatment. Conclusions: Children with DS have a significantly (1020 fold) increased risk of hematologic malignancies. But incidence of solid tumors in DS has been proposed to be lower than the general population. Only a few testicular solid tumors (mainly seminomas) were reported in DS patients in older ages ( >16 years). As it is not clear whether the incidence of testicular solid tumors are different than the normal population, we aimed to report this rare association. Keywords: Down, Malıgnancies [P-210] Phenothypic Variations in Long QT Syndrome Patients; Single Center Experience Rukiye İrem Yekeler, Elif Neslihan Tuzcu, Celal Akdeniz, Mehmet Karacan, Volkan Tuzcu ISTANBUL MEDIPOL UNIVERCITY Background&Aims: Long QT syndrome is the most common hereditary arrhytmia syndrome that can cause sudden cardiac death. With predominantly autosomal dominant inheritance, not only index cases but also asymptomatic members of the family have risk of sudden cardiac death. Same genetic mutation can lead different phenotypic presentation and QTc values, so detecting of asymptomatic carriers with EKG-QTc analysis may be incapable. Methods: Study based on regarding index cases and genetically diagnosed family members, between March 2013-November2015. With the aim of viewing phenotypic variations clearly; Jervell Lange Nielsen cases, overlap syndrome cases and LQTS cases that have more than one type of mutations are not included. Median QTc, minimum QTc, maximum QTc values are detected between family members with the same mutation. in genetically diagnosed 68 LQTS cases. OTc in index cases was 426-700 (median 472), min and max QTc values are 403-479 and 432-700 respectively. Nine of these patients were symptomatic (7 of them were index cases), 4 index cases had ICD implantation. %56.5 of these cases were LQTS 1, %17.3 were LQTS 2, %21.7 were LQTS 3, %4.3 was LQTS 8. Conclusions: Genetic test has a critical role in diagnose of hereditary arrythmia sydnromes, especially screening for asymptomatic family members. Existance of phenotypic diversity in family members having the same genotype; makes genetical test particularly important. Keywords: Long QT syndrome, genetic test, phenotypic variations [P-211] Spontaneous Pneumothorax in a Term Neonate Rukiye Taş, Tuğrul Atay, Emel Ataoğlu Department of Pediatrics, Haseki Education ve Research Hospital, ıstabul, Turkiye Spontaneous pneumothorax is a recognised and rare cause of respiratory distress in the neonatal period. Here, we report a case of symptomatic spontaneous pneumothorax associated with congenital lung disase which needed intercostal drainage. A 2760 gr, 18 days old male infant was admitted with respiratuar distress. He was vaginally delivered at term. Physical examination revealed tachypnea, subcostal and intarcostal retractions. Patient had an incision scar of a thorax tube on the left side of his chest. His medical history revealed a hospitalisation for 15 days due to spontaneous pneumothorax in neonatal period An urgent Chest X ray was ordered which revealed a left sided Pneumothorax with mediastinal shift to the opposite side. ABG showed respiratory acidosis. (pH – 7,29 PCO2 – 58 mmHg). An intercostal drainage tube was immediately placed on the left chest. Check X ray confirmed the position of the tube which functioned well. The patient was started on amphicillin and cephotaxime. CT Thorax showed left sided pneumothorax with 4 lung cyts two of which were ruptured. Since there is an association between lung cycts and cardiac and renal abnormalities, we performed echocardiography and renal ultrasonography which were normal. Antibiotics were stopped after 14 days and control X-ray showed a normal lung paranchyme, except for the 2 cysts. He has a normal growth and development with no respiratory distress. He has been on a regular follow up in chest surgery outpatient clinics since then. In conclusion, although symptomatic spontaneous pneumothorax is a rare condition in term newborns, it should always be kept in mind while evaluating a new born with severe respiratory distress for early recognition and treatment is life saving. Keywords: neonatal, Spontaneous Pneumothorax Results: Twenty two different mutations were found - 104 - [P-212] [P-213] Fatma Betül Çakır1, Rumeysa Tuna2, Ayşegül Doğan Demir2, Faruk Öktem3, Bülent Zülfikar4, Rejan Kebudi4 1 Bezmialem Vakif University, Department of Pediatric Hematology-Oncology, Istanbul 2 Bezmialem Vakif University, Department of Pediatrics, Istanbul 3 Bezmialem Vakif University, Department of Pediatric Nephrology and Rheumatology, Istanbul 4 Istanbul University, Cerrahpaşa Medical Faculty and Institute of Oncology, Pediatric Hematology-Oncology, Istanbul Background&Aims: Posttransplant lymphoproliferative disorder (PTLD) is a group of disease that occur after solid organ or hematopoietic stem cell transplantation associated with immunosuppression and Epstein Barr Virus (EBV) viral load, and characterized by atypical lymphoid and plasmositik cell proliferation. We present a successfully treated case about this rare complication of organ transplantation results in high mortality. Sairah Akbar, Hilary Pearce Royal Hospital for Sick Children, Glasgow Background&Aims: Croup is common, affecting up to 3% of children.¹ Although there is little evidence for using more than 0.15 mg/kg of dexamethasone, in practice, higher doses are often used for patients who remain symptomatic. In this audit, factors influencing the decision to give more than a standard dose were reviewed. Lymphoproliferative disorders after kidney transplant: A succesfully treated lymphoma case Methods: Six-year-old girl was diagnosed with chronic kidney failure. Cadaveric kidney transplantation was performed after two years. Recipient’s EBV viral load was negative before transplantation. After a year from transplantation under intensive immunosuppression, mass was detected with routine abdominal ultrasonography. Magnetic resonance examination showed great masses in the right quadrant of the abdomen that have made paraaortic and pelvic lymph node involvement. True-cut biopsy from the mass resulted EBV positive diffuse large B Cell Lymphoma. Results: Reducing the immunosuppressive treatment of the patient was only allowed to take steroids. After six cycles Anti-CD20 monoclonal antibody rituximab the mass decreased by 60%. Treatment with low dose chemotherapy; six cycles Vincristine and cyclophosphamide was added to therapy. At the end of this treatment 80% answer was seen. After four more cycles of rituximab complete remission was seen in the PET taken. The transplanted kidney function has remained normal. Conclusions: Lymphoproliferative disease development in pediatric patients after renal transplantation comes after heart-lung, heart and liver transplant with 2.4%. Early discontinuation of immunosuppressive therapy is the most effective treatment. Conventional standarddose chemotherapy can be mortal and monoclonal antibodies are used as first-line therapy. In patients with slow response to that therapy low-dose conventional chemotherapy can be used. Keywords: Posttransplant lymphoproliferative disorder, Renal transplantation, Epstein Barr Virus High dose dexamethasone, to give or not to give. A review of croup management in a tertiary Accident and Emergency department Methods: A retrospective audit was performed of attendances with croup between October 2013 and January 2014. Results: In total 403 patients were identified. 64% were defined as mild,² with signs present only when upset or active. 34% had moderate croup (symptomatic at rest) and the remaining 2% were moderate/severe. Ninety four percent of patients received dexamethasone. In total 19 (4.7%) were given more than 0.15 mg/kg of dexamethasone. 83% were given an additional dose or ‘top up’; the remaining 17% were given higher doses following initial assessment. When topping up, 80% were given a total of 0.3 mg/kg, 13% were given 0.6 mg/kg and 7% were given >0.6 mg/kg. The commonest reason cited was persistence of stridor in 40%; in 13% a further dose was given for worsening recession and stridor. A clear reason was not cited in the remainder. The average timing for a further dose was 2.6 hours. On further analysis, all patients given more than 0.15 mg/kg were classed as moderate croup with none of those moderate/severe given higher doses. Twenty one patients (5.2%) were given adrenaline nebulisers. Of those given nebulisers, 6 patients (29%) were given more than a standard dose of dexamethasone: 83% were given 0.3 mg/kg and one patient was given 0.6 mg/kg. Conclusions: This audit has shown variation in management for patients with croup who remain symptomatic. Further studies are recommended to determine best practice. Keywords: ‘Top up’ dose i.e. additional dose, Additional dose i.e. further dose - 105 - [P-214] [P-215] A Case Of Disseminated Fasiolazis Sare Betül Kaygusuz1, Ayşegül Doğan Demir1, Selçuk Uzuner1, Issam Cheikh Ahmad2, Özden Türel3, Faruk Öktem4 1 Department of Pediatrics,Bezmialem Vakıf University, İstanbul,Turkey 2 Department of Radiology,Bezmialem Vakıf University, İstanbul,Turkey 3 Department of Pediatric Infectious Disease,Bezmialem Vakıf University, İstanbul,Turkey 4 Department of Pediatric Nephrology,Bezmialem Vakıf University, İstanbul,Turkey Background: Fascioliasis is a trematode infection caused by Fasciola hepatica and commonly acquired by eating metacercaria encysted on leaves. İt has been reported frequently to be found from the bile ducts of patients who were operated with the diagnosis of cholecystitis. But it is often neglected despite its common occurrence in endemic areas.Here, we report a case of fasciolosis without gastrointestinal complaints who was presented as urticaria and resulting in successful treatment without any sequelae. Case: A 6 year old boy complained of swelling and redness in hands and feet for 3 months was referred to us for the evaluation of rheumatic diseases.His developmental history and physical examinations was unremarkable. Complete blood count revealed marked eosinophilia (11.760 μ/lt) which led to the suspicion of a parasitic infection. Serological examination revealed borderline titres for echinococcus.Stool exams were negative for Fasciola ova. Abdominal ultrasonography showed solid, hypoechoic lesions in the liver suspicious for malignancy.Therefore magnetic resonance imaging (MRI) of abdomen and brain were performed. Numerous hyperintense lesions in the liver were suggestive of fascioliasis.Cranial MRI revealed 4 mm subcortical hyperintense lesion in the left parieto occipital area. Fasciola antigen was found to be positive.Diagnosis of fasciolasis was based on eosinophilia, serology and mostly the characteristic findings on MRI. After the first dose of triclabendazole the symptoms disappeared. MRI scan of liver and brain done one month after therapy showed complete resolution. On follow-up fasciola antigen was found negative. Conclusions: The diagnosis of fascioliasis consists of detection of eggs in stool samples,serological tests and imaging.If detected at early stage,can be treated successfully without any sequelae. There for a high index of suspicion is important, especially because of the clinical manifestations are nonspecific and progression of the disease can cause liver failure and even death. Keywords: fascioliasis, eosinophilia, radiological diagnosis of parasitosis Henoch Schoenlein purpura on the basis of familial Mediterranean fever: Recurrent intussusception requiring surgical correction Seda Sönmez1, Seçil Arslansoyu Çamlar1, Alper Soylu1, Osman Zeki Karakuş2, Mehmet Atilla Türkmen1, Salih Kavukçu1 1 Department of Pediatrics, Medical Faculty, Dokuz Eylül University, İzmir,Turkey 2 Department of Pediatric Surgery, Medical Faculty, Dokuz Eylül University, İzmir, Turkey Introduction: Henoch Schoenlein purpura (HSP) is the most common vasculitis of childhood, and it mainly affects skin, joints, gastrointestinal tract and the kidneys. Familial Mediterranean fever (FMF) is a recessively inherited periodic fever syndrome characterized by recurrent polyserositis attacks. Several types of vasculitis are associated with FMF; the most frequent one is HSP, which occurs in about 5% of FMF patients. Case Report: An 8-year old girl presented with purpuric rash on the extensor surfaces of lower extremities and on the buttocks, arthralgia in the left knee and intermittent abdominal pain. She has been using colchicine for 4 years due to FMF which was diagnosed clinically (recurrent fever and abdominal pain episodes) and genetically (heterozygous R202Q, M680I, M694V mutations). The patient was diagnosed as HSP and oral prednisolone treatment was prescribed for abdominal pain in association with occult blood in stool. Intussusception was suspected on the basis of episodic vomiting attacks along with colicky abdominal pain, and confirmed by plain abdominal radiography and ultrasonography. Spontaneous resolution was followed by two more recurrences. The third attack did not resolve spontaneously and necessitated laparoscopic correction of both ileoileal and jejunojejunal invaginations. Discussion: Intestinal intussusception can occur in the course of HSP and is attributed to edema and damage to the vasculature of the gastrointestinal tract as a result of the vasculitic process. Intussusception occurs in 0.7– 13.6% of children with HSP. Our patient had already been diagnosed as FMF when she presented with clinical findings of HSP and invagination. However, it has been stated that the children of Mediterranean extraction who have unusual and complicated clinical picture of HSP should have genetic testing for FMF. Keywords: Henoch- Shönlein Purpura, Intussusception, Gastrointestinal İnvolvement - 106 - [P-216] [P-217] Mervan Bekdas1, Serkan Ozturk2, Buket Kara1, Seher Acar1, Hulya Ozturk2 1 Department of Pediatrics, Abant Izzet Baysal University Faculty of Medicine 2 Department of Pediatric Surgery, Abant Izzet Baysal University Faculty of Medicine Benzalkonium chloride (BAC) is a kind of skin and mucosal membranes antiseptic. Concentration used for the skin is harmful to the mucosal membranes, for this reason it must be diluted. In this case study, an infant with severe esophagitis after ingestion of BAC is reported. A 2 months old male patient was admitted to hospital for fever after vaccination. Paracetamol suspension was proprosed, but the baby’s mother was administered her baby with 5 ml of 10% BAC (zephiran TM) instead of paracetamol accidentally. Agitation and vomiting began after taking it. The baby was referred to our hospital. On admission, he had a pulse rate of 168 bpm, blood pressure of 80/58 mmHg, respiratory rate of 62/min and body temperature of 37 °C. On physical examination of the unrestlesness patient, hyperemia and increased salivation were detected in oral mucosa and pharynx, the other systems findings were unremarkable. The hemogram revealed hemoglobin: 9.9 g/L, hematocrit: 27.5%, WBC: 9.9x109/L, and platelets:447x109/L. Serum chemical values were within the reference range, fecal occult blood was negative. There was no abnormal finding on the chest radiography and arteriel blood gas values. Because of the edematous epiglottis and vocal cords, endotracheal tube was inserted, assisted ventilation (PEEP:5, PİP:20) was initiated. An endoscopic examination, mucosal edema, hyperemia and fibrin plaques in esophagus were revealed. Enteral feeding was stopped. Total parenteral nutrition, sulbactamampicillin 100 mg/kg, amikacin 15 mg/kg of amikacin, metilprednisolone 2 mg/kg and ranitidine 3 mg/kg was begun. He was extubated and enteral feeding was started on the fifth day. All medications were stopped on the eighth day and discharged in the same day. Esophageal stenosis was not detected on contrast radiography which made at the end of the third week. Consequently, parents should be very careful while giving medication to children. Keywords: Benzalkonium, esophagus, child Fatih Demircioglu1, Bengu Ulku Tarhan2, Mervan Bekdas1, Mustafa Erkocoglu1, Sevil Bilir Goksugur1, Mustafa Dilek1, Seher Acar1, Elifnur Ildes1 1 Department of Pediatrics, Abant Izzet Baysal University Faculty of Medicine 2 Department of Pediatrics, Duzce University Faculty of Medicine Infectious mononucleosis (IM) is presented as adenopathies in the neck, swallowing difficulty and fever. Mild thrombocytopenia accompanies approximately more than 50% of patients with IM. Pseudothrombocytopenia (PT) is a phenomenon in which a low platelet count is due to the presence of antiplatelet antibodies in blood that cause platelet clumping. It may be seen in normal individuals or some disorders when their blood samples are anticoagulated in a medium containing ethylenediaminetetraacetic acid (EDTA). A 9 year old male patient was admitted to our outpatient clinics for the assessment of fever and neck adenopathies. His parents denied any bleeding disorder like epistaxis, melena, hematuria and easy bruising. His platelet count was 36.000 /mm3 by electronic counting method. Microscopic examination of peripheral blood smears showed adequate numbers of platelets and reactive lymphocytes typical of IM. Then, the diagnosis of IM was confirmed by the presence of IgM against Ebstein barr virus. In addition, when the blood count was processed in a medium containing citrate instead of EDTA, the platelet count was 152.000/ mm3. PT is a condition in which the risk of bleeding, stroke or the need for platelet transfusion are not increased. It can be associated with many diseases. EDTA dependent PT is the most frequent cause of PT. The misdiagnosis of the thrombocytopenia can be prevented with the microscopic inspection of blood smear. The finding of PT prevents unnecessary patient examinations and therapeutic interventions. In our case, the cause of PT was IM. False severe thrombocytopenia is seen in this case and the possibility of these situations must be taken into account to avoid misdiagnosis and mismanagements. Keywords: Pseudothrombocytopenia, Infectious Mononucleosis A bad result of carelessness: Esophageal necrosis due to benzalkonium chloride ingestion An Atypical Cause Of Pseudothrombocytopenia; Infectious Mononucleosis [P-218] A Rare Diagnosis In Neonatal Period: Lipoprotein Lypase Deficiency Selen Baran1, Ertuğrul Kıykım2, Tanyel Zübarioğlu2, Ayşe Çiğdem Aktuğlu Zeybek2 1 Istanbul University, Cerrahpasa Medical Faculty, Department of Pediatrics, Istanbul, TURKEY 2 Istanbul University Cerrahpasa Medical Faculty, Department of Pediatrics Division of Nutrition and Metabolism, Istanbul, TURKEY Lipoprotein lypase deficiency ( hyperlipoproteinemia type 1 ) is a rare disorder which is inherited in an autosomal recessive fasion. The course of the disease consists of hyperlipidemia, chylomicronemia and related - 107 - clinical symptoms. This condition is mostly diagnosed during childhood period with hepatosplenomegaly accompanied with abdominal pain, pancreatitis attacks and xanthomas. It is rarely diagnosed during adulthood. Here we present a 5 days-old neonate who was referred to our out-patient clinic with neonatal hyperbilirubinemia. Her blood samples were lypemic and laborotory tests revealed triglyceride levels as 5540 mg/ dl and total cholesterol levels as 426 mg/dl. According to the results apo-c2 deficiency and lipoprotein lypase deficiency considered in the differential diagnosis. We performed 5 days of plasma replacement therapy which consist of apo-c2, however laboratory results did not decrease. Afterwards, 5 days of diet resulted in a marked decrease in triglyceride and total cholesterole levels which revealed lipoprotein lypase deficiency. Lipoprotein lypase deficiency is rarely diagnosed in neonatal period because symptoms can usually be obvious after infancy. Coincidental diagnosis of lipoprotein lypase deficiency in this case can make our patient have less secondary complications of the disease because of the early treathment. Keywords: Lipoprotein lypase deficiency, hypertriglyceridemia [P-219] A Not Fully Vaccinated Case:Whooping Cough Sema Bozkaya Yilmaz1, Erdal Sari2, Feyza Mediha Yildiz3, Rumeysa Citli4, Handan Akoglu5 1 Sema Bozkaya Yilmaz,Zeynep Kamil Maternity And Childrens Diseases Training And Research Hospital,İstanbul,Turkey 2 Erdal Sari,Zeynep Kamil Maternity And Childrens Diseases Training And Research Hospital,İstanbul,Turkey 3 Feyza Mediha Yildiz,Zeynep Kamil Maternity And Childrens Diseases Training And Research Hospital,İstanbul,Turkey 4 Rumeysa Citli,Zeynep Kamil Maternity And Childrens Diseases Training And Research Hospital,İstanbul,Turkey 5 Handan Akoglu,Zeynep Kamil Maternity And Childrens Diseases Training And Research Hospital,İstanbul,Turkey Introduction: Whooping cough is caused by gram negative pleomorphic bacillus,Bordatella Pertussis.It is a highly contagious and potentially life-threatening respiratory tract illness. The illness can be diagnosed by culture or PCR on a properly collected nasopharyngeal swab.Here, we present a case of pertussis who was unvaccinated because of her special condition. Case: A 2-year-old female patient admitted to our pediatric clinic with persistant cough with paroxysyms lasting for 2 weeks.The patient presented to another clinic 1 week ago with the same complaints and was treated with salbutamole nebules.She was not fully vaccinated because of her atopic dermatititis. She was admitted to hospital;chlarithyromycin and nebulized budesonide therapy was started.Blood and nasopharyngeal swab samples were tested for pertussis.After seven days of hospitalization,her symptoms resolved. Nasopharyngeal swab was positive for Bordatella Pertussis. Discussion: Isolation of B.Pertussis in culture is the gold standard for diagnosis.Direct fluoroscent antibody testing of potential isolates using specific antibody against B.Pertussis and B. Parapertussis maximizes detection rates. Polymerase chain reaction analysis to test nasopharyngeal wash specimens has a sensitivity similar to that of culture and averts difficulties on isolation. Unvaccinated refugees and other special conditions resulting with unvaccination or delaying vaccination may precipitate rare diseases.We present this case to remind you pertussis in case of chronic cough,which is a rare condition in fully vaccinated communities. Keywords: Pertussis, Vaccination [P-220] Intracranial Hemorraghe With Herpes Simplex Virus Encephalitis: A Case Report Sermin Özcan, Ihsan Kafadar, Haşim Gencer, Nazan Dalgıç Şişli Hamidiye Etfal Eğitim ve araştırma Hastanesi, Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalı, İstanbul Herpes Simplex Virus (HSV)is the main cause of sporadic viral encephalitis. HSV is characterized by causing necrotizing hemorrhagic encephalitis on medial temporal and frontal lobes. It is documented that HSV cause intracerebral hemorrhage in infants and also in early childhood. Virus causes encephalitis by spreading to brain via peripheral nerves like trigeminal and olfactory nerves. Symptoms like headache, fever, seizure, unconsciousness develop within a few days. Early diagnosis and acyclovir treatment may reduce the mortality to %19 whereas the mortality rate is approximately %70 in untreated child patients. Herpes simplex encephalitis (HSE) is the most common cause of fatal acute sporadic encephalitis in older than 6 months children and adults. HSV has a ratio of %2-19 in whole encephalitis cases wheras it has a ratio of %20 -75 in whole necrotizing encephalitis cases. We prepared this case to emphasize the importance of thinking HSE as diagnosis in febrile encephalopathy patients with focal neurologic signs with the acknowledgment of early diagnosis and treatment’s positive effect on mortality, also to determine problems encountered and the points that need attention at diagnosis of HSE. Keywords: herpes simplex virus, encephalitis, intracranial hemorrhage [P-221] Congenital Nephrogenic Diabetes Insiipidus Sermin Özcan, Nurver Akıncı, Zeynep Civelek, Merve Şakar Şişli Hamidiye Etfal Eğitim ve araştırma Hastanesi, Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalı, İstanbul Introduction: Congenital (primary) Nephrogenic Diabetes Insipidus is a rare inherited disorder - 108 - characterized by insensitivity of distal renal tubule to the effect of antidiuretic hormone (ADH), which may cause growth and mental retardation. The clinical symptoms can be different and hard to diagnose in infants and newborns relatively older children. Case: A-32 days old boy was brought pediatric emergency room with fever, feeding problems and irritability. In anamnesis, polyuria and polydipsia were present in mother and mother’s family. In physical examination lack of affinity to allimentation and affinity to water were observed. 38.2°C of body temperature, dry skin and sunken eyes were all observed pathological findings. Hypernatremia (Na:157mmol/l) and low density of urine (d:1001) were determined in blood and urine tests. After hydration due to the hypernatremic dehidration prediagnosis, high blood sodium level and low urine density were obtained. Hydration was cut, and baby was started to feed with only alimentaion, without extra water. However high sodium level, dehydration and the low urine density continued. His urinary and cranial imagings were checked and no pathological evidences were found. Desmopressin 6mg/kg/day was given as a treatment and a small increase was obtained in urine density however hypernatremia and polyuria continued and central cause of diabetes insipidus was put a side. Indometasine 2mg/kg/day was started and labatory test results bacame normal and polyuria stopped. It was understood that the reason of diabetes insipidus was nephrogenic causes. Result: It is aimed with this case, Congenital Nephrogenic Diabetes Insipidus should be the differential diagnosis of the patients with hypernatremic dehidration. Keywords: diabetes insipidus, hypernatremic dehydration, congenital [P-222] Association Between Urinary Bisphenol A Levels And Body Weight Among Children In Our Clinic Sevinç Gümüş1, Ayşe Kılıç1, Ismet Çok2, Dilek Battal3, Şükran Poyrazoğlu4, Feyza Darendeliler4 1 Istanbul University, Istanbul Faculty of Medicine, Department of Pediatrics, Istanbul, Turkey 2 Gazi University, Faculty of Pharmacy, Pharmaceutical Toxicology, Ankara, Turkey 3 Mersin University, Faculty of Pharmacy, Pharmaceutical Toxicology, Mersin, Turkey 4 Istanbul University, Istanbul Faculty of Medicine, Department of Pediatric Endocrinology, Istanbul, Turkey Background And Aims: Prevalence of childhood obesity is rising in which endocrine disruptors chemicals play an important role. Bisphenol a is one of them widely used in food and drink packaging. It mimics estrogen that leads to insuline resistance and obesity. We aimed to draw attention on this topic and collect data about Turkish children. Methods: Our study group is consisted of children among patients followed up with overweight and obesity in Istanbul Faculty of Medicine, Pediatric Endocrinology outpatient clinic. Normal weight group is selected among children applied to Istanbul Medical Faculty General Pediatrics outpatient clinic with similar age and sex distribution. Weight and height were measured by trained personnel. Fresh spot urine samples preserved in glass tubes at -20Cjavascript:addsym(‘%C2%B0’); until laboratory testing. Urinary bisphenol A (BPA), bisphenol A glucronid (BPAG) and creatinine levels are mesured with liquid chromatography–tandem mass spectrometry. Results: 172 participants were %47,7 (n=82) male, %52,3 (n=90) female who aged between 4 and 18 (mean 11,68±3,25) years. Mean BPA and BPAG concentration corrected with urine creatinin is 182,46±293,18 mg/g creatinin in general, 138,46±224,08 in obese, 105,91±174,95 in overweight, 104,36±127,80 in normal weight group. BPA concentrations revealed poor correlation with Body mass index (BMI) but no statistically proven difference among the groups. When participants were grouped by BPA concentration persantile obesity prevalence did not show difference. Conclusions: Factors related to obesity are not easy to study as other multifactorial diseases. Although it is not proven statistically we had a clue about relation of BPA an childhood obesity. Endocrine disrupters is an important topic needed to be focused on. We think that this study is important to be the beginning point and nationally representative study is needed to evalute the effects of BPA in Turkish population. Our study is financially supported by Istanbul University Scientific Research Projects Coordination Unit. Keywords: endocrine disruptors, bisphenol a, childhood obesity [P-223] Unnecessary Antibiotic Use in Hand Foot and Mouth Disease Sule Nursah Ayata1, Burcu Karadaş2, Hatice Dülek1, Hüray Kök2, Zeynep Emine Tuzcular Vural1, Çağatay Nuhoğlu2 1 Department of Family Medicine, Haydarpasa Numune Training And Research Hospital, Istanbul, Turkey. 2 Department of Pediatrics, Haydarpasa Numune Training And Research Hospital, Istanbul, Turkey. Background&Aims: Hand, foot, and mouth disease (HFMD) mostly affects infant and young children and is one of the major public health concerns in developing countries. Characteristic clinical signs and symptoms are usually sufficient to diagnose the disease and no specific treatment other than relief of symptoms is necessary in usual cases. High rate of unnecessary antibiotic use is remarked in this case series. Methods: Clinical data of 35 children were retrospectively reviewed in Haydarpaşa Numune Training and research Hospital, Department of Pediatrics. Hematological or serologic investigation has not been performed and diagnosis was made according to clinical and physical examination of the patients, which are mild fever, fatigue, odynophagia, rash on the hands and feet, and blisters in the oral cavity. Results: Among the 35 children, 23 (65.7%) were boys - 109 - and 12 (34.3 %) were girls, and their mean age was 20.4 ± 14.0 months. Majority of the patients were between 3 and 20 months of age, which accounted for 60% (21 of 35) of the total patients. Rash was the most common symptom (35 of 35 patients) and was the major reason for parents to seek medical care. Five of the patients (14.3%) were hospitalized overnight for treatment of dehydration and rest of the patients were given outpatient care. Before admission to our clinic 15 of patients (42.9 %) had been prescribed antibiotics by pediatricians or primary care physicians due to misdiagnosis or fear from superinfection. Conclusion: In this case series almost half of the patients had been treated with antibiotics for some reason. In this report we remark the the importance of being familiar with the specific clinical scene of HFMD for pediatricians and primary care physicians to making the correct diagnosis and avoiding unnecessary antibiotic treatments. Keywords: Rash, Hand Foot and Mouth Disease, Antibiotic Treatment [P-224] Pediatric Mastocytosis: Report of 7 cases Süheyla Ocak1, Esra Yücel2, Hilal Susam Şen1 1 Okmeydani Research and Training Hospital, Department of Pediatric Oncology 2 Okmeydani Research and Training Hospital, Department of Pediatric Allergy Background&Aims: Pediatric mastocytosis can very easily be mistaken for a variety of common rashes that plague the pediatric population.The management of children with mastocytosis possess a chalenge as due to lack of evidence that reliably predict prognosis. Here we presents 7 cases of mastocytosis diagnosed between November 2014- June 2015. Results: Of the 6 children with mastocytosis and 1 child with mastocytoma 5 were male and 2 were female. The mean age at first symptom and at diagnosis were 4 months (range 1-6 months) and 11,5 months ( range 6-22 months), respectively. Skin rash is the main complaint. All patients were evaluated by a general pediatrician at first admission, and the major diagnoses were allergic eruption and dermatitis. The diagnosis was proved by skin biopsy in all cases. On admission aside from the diffuse,multiple, solmon or pink coloured eruption, all patients had normal physical examination. Complete blood counts with differential, peripheral blood smear, renal and liver function tests, serum IgE and tryptase levels were all normal. Abdominal ultrasonography did not suggest systemic involvement. Oral antihistaminics and mast-cell stabilizers were prescribed for symptomatic patients. Anaphylaxis did not ocur in any patients. Although the number and the site of the lesions have changed with time, no spontaneous disappearance were documented on mean follow-up period of 8 months (range 4-12 months). to differentiate from other common causes of skin eruptions in infants and to provide symptomatic relief to patients. Also informing the parents about the follow-up and prognosis will prevent anxiety and the uncessary visits to hospital. Keywords: child, cutaneous mastocytosis [P-225] Pneumonia Is More Than Pneumonia Şifa Şahin1, Tuba Koçkar1, Halil Suat Ayyıldız2, Sedat Öktem1, Ömer Ceran1 1 Pediatrics, Faculty of Medicine, Medipol University,Istanbul Turkey 2 Pediatric Surgery, Faculty of Medicine, Medipol University,Istanbul Turkey Introduction: Pneumonia is the most cause of morbidity and mortality of childhood. Generally infiltrations of X-ray make a sign infections of lung like pneumonia. Therefore other reasons are disregarded for many times. Clinicians have to consider other causes for differential diagnosis. Case 1: The patient was 6 year old girl with a history of fever, cough and persistant hemoptysis. And chest X-ray infiltration was determined. She had treated with antibiotics for three times as a pneumonia. Hemoptysis persisted although treatment. The patient was excamined again and properly limited round infiltrate area was detected on right hemithorax pericardial area. Serological tests were examined for hydatid cyst and treated with albendasole. Hemoptysis stopped. Figure.1 Case2: 6 year old boy, had suddenly onset and persistent cough with fever. An infiltration was noticed on left hemithorax and the patient was treated with broad spectrum antibiotics. But symptoms persisted. After an evaluation of history and the image of chest, we thought that it may be foreign body aspiration. We performed bronchoscopy and peanut was detected left main bronchus. Figure.2 Case 3: 3 month year old boy, the patient had fever, cough, desaturation from the birth. There was no feeding intolerance. And the patient was treated with antibiotics for many times. He was unresponsive to treatment. We saw an air density on right hemythorax on chest X-ray. We performed esophagography and hiatal hernia was detected. Figure.3 Case4: 3 year old girl who had neurological defect, was treated as a pneumonia for many times. But we considered another diagnosis for recurrent pulmonary infections. So that we determined swallow disfunction. Conclusion: All infiltrations are not always pneumonia. Physicians should consider other reasons like cyst hydatic, swallow disfunction, foreign body aspiration, hiatal hernia. Keywords: Pneumonia, cyst hydatic, hiatal hernia Conclusions: Pediatric mastocytosis is a rare and pediatricians is not familiar with the disease. Although it is mostly limited to skin, early recognition is important - 110 - [P-226] A Case Of CHARGE Syndrome With Recurrent Respiratory Problems Hatice Ütkü Sınav1, Şifa Şahin1, Leyla Telhan2, Fatma Gamze Demirel3, Sedat Öktem4 1 Pediatrics, Faculty of Medicine, Medipol University, Istanbul Turkey 2 Pediatric Critical Care Medicine,Faculty of Medicine, Medipol University, Istanbul Turkey 3 Pediatrics Neonatalogy, Faculty of Medicine, Medipol University, Istanbul Turkey 4 Pediatric Pulmonology, Faculty of Medicine, Medipol University, Istanbul Turkey Introduction: The estimated birth incidence of CHARGE syndrome is 1 in 8.500 -12.000. This syndrome is generally associated with coloboma, heart defect, choanal atresia, retarded growth and development, genital hypoplasia, ear anomalies/deafness. Children with CHARGE syndrome require multidisciplinary evaluation and some times need pediatric intensive care follow-up. Case: 26 week gestational age, 620 gr male baby was born by cesarean section to a 24-year old female. The infant had Apgar scores of 1 and 7 at first and fifth minutes. He was intubated and transferred to neonatal intensive care unit for assisted ventilation. He had dismorphic face appearance, ear anomaly. We could not pass through nasogastric tube on right side of nasal cavity. Choanal atresia was determined on computed tomography and treated surgically. Echocardiography was performed and of bronchopulmonary dysplasia. He was discharged with home ventilation. However he hospitalized many times for aspiration pneumonia outpatient follow up. We examined etiology of aspirations. Gastroesophageal reflux determined. It was applied Nissen fundoplication, gastrostomy and symptoms decreased. Genetic tests did not result yet. Patients’ follow-up still continue in pediatric pulmonology. Conclusion: CHARGE syndrome is a rare disease and a clinical diagnosis. The etiology may be genetically as with other syndromes. Clinicians should consider this syndrome for patients that have multi system defects and recurrent respiratory problems and aspirations. Keywords: CHARGE syndrome, coloboma, choanal atresia [P-227] Statistical analysis of the Syrian mothers and their babies in our hospital Tolga Besci1, Özge Besci1, Zeynep Alp Ünkar2, Aslı Memişoğlu2, Hülya Özdemir2, Hülya Bilgen2, Eren Özek2 1 Derpartment of Pediatrics, Marmara University Medical Faculty, İstanbul, Turkey 2 Division of Neonatology, Derpartment of Pediatrics, Marmara University Medical Faculty, İstanbul, Turkey Background & Aims: Immigration may result in deaths, spreading contagious diseases, inclining poverty, malfunctioning health issues related to mother and child, antenatal care and family planning. Immigrant mothers’ difficulties to reach for antenatal care, mortality of the fetuses, the incidence of low birth weight infants, the rate of birth of the premature infants and the prevalence of infectious diseases are discussed in different researches worldwide. We intended to outline the demographical characteristics and the health issues related to the hospital stay of the Syrian mothers and their babies who applied to our hospital in year 2014. Methods: Demographical data, and problems related to the hospital stay of the pregnant women and their babies in our hospital in year 2014 were retrospectively analyzed from the patients’ files. Results: In year 2014 in our hospital, 72 (%2.6) of all the 2753 live births were Syrian refugees. Only 57 of 72 mothers’ pregnancy history could be taken. Mean mother age was 24.3±6.4 years. Adolescent pregnancy rate was (<19years) %19.4. Average pregnancy number was 2.6. Average gestational age of the babies was 37.5±3.2 and the average birth weight 3068±705 gr. Of all babies %5 was preterm, %12.5 was late preterm, 7% of the babies of small for gestational age. 7% of all the babies needed resuscitation at birth. 12 % of the babies were internalized in neonatal intensive care unit (NICU). Conclusions: Communication difficulties with the mothers create problems in defining the babies at risk. Delay in diagnosis and/or treatment could be an inevitable result of an inaccurate history. Despite the language problem, fewer babies than expected were admitted to NICU with the help of effort and good communication skills of health care providers. Thus, in order to improve the quality of health care services given to immigrants, more translators are needed in hospitals. Keywords: Syrian refugees, health problems, maternity [P-228] A Case Report: Haemophagocytosis with Respiratory Distress and Lymphangiomatosis with Bleeding Tuba Koçkar1, Pınar Yamaç2, Ebru Tuğrul Sarıbeyoğlu3, Suat Ayyıldız4, Rıza Rızalar4, Sedat Öktem1 1 Pediatric Pulmonology, Faculty of Medicine, Medipol University, Istanbul, Türkiye 2 Pediatrics, Faculty of Medicine, Medipol University, Istanbul, Türkiye 3 Pediatric Haemotology, Faculty of Medicine, Medipol University, Istanbul, Türkiye 4 Pediatric Surgery, Faculty of Medicine, Medipol University, Istanbul, Türkiye Background: Lymphangiomatosis is a syndrome in which multiple organs are involved. Frequent anatomic sites of involvement include the mediastinum, retroperitoneum, axilla and neck. Lymphangiomatosis is usually fatal at an early age. Objection: We aimed to highlight a rare causes of inspiratory stridor and respiratory distress in children before age 2. - 111 - Case: A 21 months of girl was admitted with respiratory distress, malaise and failure to thrive. She had story of long stay at hospital in Georgia. She was performed several laboratory test and examination but her diagnosis were not named yet. Sweat chloride test result was found intermediate levels and genetic analysis resulted 7T/7T polimorfism (which is nonsense mutation for cystic fibrosis). So that she referred to our hospital suspicion of cystic fibrosis. There was shortness of breath, inspiratory stridor, several ecchymosis on trunk and extremity, bilateral multipl servical lympadenomegaly, abdominal distension, hepatomegaly and splenomegaly in her first examination. Abdominal ultrasonography confirmed an increase in size of the spleen and liver; the spleen size was 120 mm and the liver size was 100 mm. Servical ultrasonography confirmed in various size of servical and submandibular multipl lympadenomegaly. Abdominal computed tomography confirmed hepatosplenomegaly and probably suggested diffuse lymphangiomatosis. Oral bleeding and epistaxis started and progressively increased then dissemine intravascular coagulation occurred. She was transfused many times with erytrocyte and thrombocyte suspensions, fresh frozen plasma. She underwent palliative splenectomy. The pathologic findings suggested that massive splenomegaly (the size of spleen was from 6.5 cm to 16 cm. The pathologic diagnosis was haemophagocytosis and lymphangiomatosis. Conclusion: Diffuse lymphangiomatosis is a rare condition in infancy. It presented various symptoms. Pediatricians should be vigilant lymphangiomatosis for in patients with inspiratory stridor beside servical and mediastinal vascular mass especially in infancy and early childhood. Keywords: Infancy, Stridor, Lymphangiomatosis [P-229] An Alternative Treatment to Tracheostomy in Pierre Robin Sequence: Mandibular Distraction Osteogenesis Tuba Koçkar1, Sedat Öktem1, Ismail Mithat Akan2, Ayhan Taştekin3 1 Pediatric Pulmonology, Faculty of Medicine, Medipol University, Istanbul, Turkey 2 Plastic Surgery, Faculty of Medicine, Medipol University, Istanbul, Turkey 3 Neonatology, Faculty of Medicine, Medipol University, Istanbul, Turkey Background: The triad of glossoptosis, micrognathia, and cleft palate characterizes the Pierre Robin sequence (PRS). Mandibular distraction osteogenesis can be a safe and effective way to avoid tracheostomy placement in selected neonates with Pierre Robin sequence. Case: A two month-old boy who was admitted to the hospital with the complaints of cyanosis and feeding difficulty and he was diagnosed as Pierre Robin sequence. He had respiratory distress after a while deteriorated. Firstly he followed with intermittent nasal continuous positive airway pressure (CPAP) then underwent assisted ventilation. He had difficulty in weaning from mechanical ventilation so that flexible bronchoscopy was performed for evaluation of respiratory tract. We established severe laryngomalacia. Nasopharyngeal airway was performed to the patient to ensure airway safety. But respiratory distress did not improve, therefore we decided to perform mandibular distraction osteogenesis and tracheostomy. In follow up difficulty of feeding was improved after 11th day of mandibular distraction osteogenesis. Computerized tomography revealed an adequate airway; therefore tracheostomy was closed at 18th day. The distractors were removed two months after ossification of callus formation. We did not observe any complications during the surgery or post-operative follow-up visits. Conclusion: Mandibular distraction osteogenesis can be used as an alternative treatment in Pierre Robin syndrome patients who underwent tracheostomy. A successful external mandibular distraction induces the removal of the airway obstruction, allowing spontaneous ventilation without devices. Keywords: Pierre Robin, Mandibular Distraction Osteogenesis [P-230] A Case of Pulmonary and Intracardiac Thrombosis in Behcet Disease Tugce Goksu Yilmaz, Fatih Mehmet Keleşoğlu, Gafur Doğdu, Ayça Gürbüzer, Hakan Çakır, Sevinç Emre, Rukiye Eker Ömeroğlu, Başak Saraçoğlu Istanbul University, Istanbul Faculty of Medicine Case Presentation: The present report is of a 4-year-old male patient, admitted to the Istanbul University Istanbul Faculty of Medicine Pediatric Emergency Department with complaints of cough and hemoptysis. The patient had been admitted to various centers with complaints of chest pain for the past two months. Patient’s symptoms including hemoptysis did not improve with treatment, then we decided to do further work-up for diagnosis. Thorax CT scan showed thrombi in both lungs and heart. Thrombus in the right ventricle was demonstrated with Echocardiography and Cardiac Magnetic Resonance Imaging (Figure 1). The patient was hospitalized for further work-up and treatment. Department of Pediatric Hematology was consulted. Bloodwork was ordered to investigate the etiology of thrombosis and Low Molecular Weight Heparin (LMWH) was started following the suggestions of the Department of Pediatric Haematology. Further anamnesis revealed history of recurrent oral aphtosis. Due to the fact that Behçet’s disease could present with trombi in similar locations, Pathergy test was ordered. Pathergy test was negative but HLA B51 test was positive. The patient was given intravenous methylprednisolone and azathioprine therapy. Complaints of chest pain and hemoptysis responded well to the therapy. Intravenous methylprednisolone was later replaced with oral prednisone which was tapered off. The patient is now only on azathioprine and followed-up in the outpatient clinic. Keywords: Behcet’s, Disease, thrombosis - 112 - [P-231] [P-232] Tugce Kalayci Oral1, Nazli Kavcik1, Ilker Gonen1, Elif Kirit1, Esin Aldemir2, Aysel Kiyak3, Sevgi Yavuz3 1 Department of Pediatrics, Kanuni Sultan Suleyman Education and Research Hospital 2 Department of Neonatology, Kanuni Sultan Suleyman Education and Research Hospital 3 Department of Pediatric Nephrology, Kanuni Sultan Suleyman Education and Research Hospital Introduction: Nephrogenic diabetes insipidus (NDI), is a group of diseases caused by decreased urine concentration capability due to antidiuretic hormone (ADH) insusceptibility, resulting in polyuria and polydipsia. Vesicoureteral reflux disease (VUD) and ureterovesical junction (UVJ) obstruction are congenital urinary tract abnormalities and coexistence of those is very rare. In this paper, we would like to present a case, diagnosed with nephrogenic diabetes insipidus secondary to stage 5 right VUD and left UVJ obstruction. Tugce Kalayci Oral1, Sevgi Yavuz2, Aysel Kiyak2, Hamide Sevinc Genc1, Mustafa Cakan3, Nuray Aktay Ayaz3 1 Department of Pediatrics, Kanuni Sultan Suleyman Education and Research Hospital, Istanbul, Turkey 2 Department of Pediatric Nephrology, Kanuni Sultan Suleyman Education and Research Hospital, Istanbul, Turkey 3 Department of Pediatric Rheumatology, Kanuni Sultan Suleyman Education and Research Hospital, Istanbul, Turkey Introduction: Cystinosis is an autosomal recessively inherited disease characterized by accumulation of cystine in tissues due to transporting defect from lysozymes. It accumulates in cornea, bone marrow, thyroid gland, pancreas and mainly in kidney and causes organ dysfunction. Involvement of skeletal system is a very rare entity. Until this date, only one patient with cystinosis reported to have arthritis. We would like to present a case, applying to our outpatient clinic with arthritis and diagnosed with cystinosis. Nephrogenic diabetes insipidus with the background of vesicoureteral reflux disease and ureterovesical junction obstruction A Cystinosis Case Presenting With Arthropathy Case Presentation: A patient, 4 days old, applied to emergency clinic with fever. He had antenatal diagnosis of left renal hydronephrosis. Weight loss detected as 360 grams (%13). The patient was admitted to NICU with pre-diagnosis of hypernatremic dehydration. Even though adequate hydration was obtained; urea, creatinine, Na ve Cl levels preceded as high and urine density as low. Polyuria was constant. “Bilateral severe hydronephrosis” was found in abdominal ultrasound. Voiding cystourethrography showed stage 5 right VUR. MAG-3 scintigraphy was concordant with left UVJ obstruction. Patient diagnosed as nephrogenic diabetes insipidus. Hydrochlorothiazide treatment started. Result: NDI develops due to ADH insusceptibility of kidneys. NDI patients are under risk for neurodevelopmental retardation, due to hypernatremic dehydration. In our patient, uncorrectable hypernatremia by hydration, stage 5 VUD in right kidney and left UVJ obstruction led us to diagnose him as secondary nephrogenic diabetes insipidus and normal ADH values helped us to differentiate this disease from central diabetes insipidus. The main goals of the treatment are replacing urinary water loss with appropriate fluids and decreasing urine output. Urine output can be decreased by medical treatment up to %30-70, daily. As a result, screening renal pathologies in children applying with hypernatremia, hypercholoremia and dehydration; although rare, keeping in mind nephrogenic diabetes insipidus as secondary to those pathologies is important in decreasing treatable complications such as growth and mental retardation. Keywords: NDI, VUD, UVJ Case Presentation: A 3 year old patient applied to our outpatient clinic with the complaints of pain and swelling of the right knee for 2 weeks. In her history, polyuria, polydipsia and lack of weight gaining were present. The parents did not describe a recent infection or fever. Weight and height were found to be below 5 percentile, body temperature was 36.5 C and blood pressure was 90/50 mmHg. Her physical examination revealed sensitivity, swelling and lack of movement in the right knee. There was no significant elevation of temperature. Other system examinations were normal. In her laboratory findings, full blood count and acute phase reactants were in normal ranges but hypokalemia, hypophosphatemia, mild metabolic acidosis, glycosuria, proteinuria and hypercalciuria were detected. Increased joint fluid of the right knee found in radiological evaluation. The patient started on ibuprofen and arthritis findings are regressed in two weeks. Acute phase reactants remained in normal ranges. Her wrist x-ray revealed findings of rickets and ophthalmologic examination showed cystine crystals. Her blood leukocyte cystine levels found to be high, she was diagnosed as cystinosis and started on cysteamine treatment. Discussion: This case emphasizes the importance of careful evaluation of renal tubular functions in patients with arthritis accompanied by growth retardation, rickets and electrolyte imbalance; and keeping cystinosis in mind in differential diagnoses Keywords: cystinosis, arthritis, cystine - 113 - [P-233] The Relationship Between Streptococcal Tonsillopharyngitis and Erythema Nodosum in a Child [P-234] Five Children with Leg Pain Tuğçe Kalın1, Aysun Yahşi2, Tuğçe Tural Kara2, Döndü Nilay Penezoğlu1, Osman Umur Başaran1, Çisem Saygılı3, Tuğba Erat2, Halil Özdemir2, Ergin Çiftçi2, Erdal İnce2 1 Department of Pediatrics, Ankara University Medical School, Ankara, Turkey 2 Department of Pediatric Infectious Diseases, Ankara University Medical School, Ankara, Turkey 3 Department of Family Medicine, Ankara University Medical School, Ankara, Turkey Erythema nodosum (EN) is a variant of septal panniculitis with no vasculitis which occurs most probably due to the antigenic stimuli by inciting factors like infections, sarcoidosis, rheumatologic diseases, inflammatory bowel diseases, medications, autoimmune disorders. Streptococcal infections are the most frequently causative factors in children; the cutaneous lesions appear 2-3 weeks after the throat infection. Tissue defect is localized on the anterior aspects of the legs which is caused by reactive oxygen intermediates produced by activated neutrophils and the subcutaneous fat are infiltrated by inflammatory cells. EN recovers without scarring and ulceration in 3-6 weeks spontaneously. Bed rest, anti-inflammatory drugs, potassium iodide, systemic corticosteroids, colchine, hydroxchloroquine can be used for treatment. Herein, we reported a 10year old-girl who had EN secondary to streptococcal tonsillopharyngitis. She was suffering from painful, edematous and erythematous lesions localized on bilateral anterior tibia. Her complaints started two weeks ago. Fever and cough added at the same time. On the physical examination, she had oropharynx hyperemia and the typical eruption of EN that included symmetric, tender, erythematous, warm and raised plaques located on her knees. Group A β-hemolytic streptococcus (GABHS) was isolated from the throat culture. Following the laboratory examinations, hemoglobin: 11.1 g/dL, leukocyte count: 19340/mm³, platelet count: 506000/ mm³, sedimentation: 74 mm/h, C reactive protein: 38.6 mg/L, antistreptolysin O titer: 2864 IU/mL. Biochemical parameters and radiograph were normal. Additionally, PPD test was negative. She was diagnosed as EN secondary to GABHS. Oral paracetamol and intravenous methylprednisolone were given for 3 days. As expected, clinical findings were regressed, she was discharged with oral methylprednisolone. As a result, EN, which is an unusual cutaneous reactive process in children, may be seen as a complication of streptococcal infections. Because of this, GABHS should be considered as a causative agent of EN. Keywords: childhood, erythema nodosum, streptococcal tonsillopharyngitis Tuğçe Kalın1, Tuğçe Tural Kara2, Halil Özdemir2, Fatih Günay1, Nisa Eda Çullas İlarslan1, Elif İnce3, Deniz Tekin4, Zümrüt Uysal3, Emine Zeliha Suskan4 1 Department of Pediatrics, Ankara University Medical School, Ankara, Turkey 2 Department of Pediatric Infectious Diseases, Ankara University Medical School, Ankara, Turkey 3 Department of Pediatric Hematology and Oncology, Ankara University Medical School, Ankara, Turkey 4 Department of Pediatric Emergency, Ankara University Medical School, Ankara, Turkey Leg pain, which has so many different causes, is a common complaint in childhood. The pediatricians should always be skeptical when evaluating the leg pain and take a detailed history, make physical examination carefully. When an underlying disease is suspected, laboratory tests and imaging should be done. In this article, we studied five cases with leg pain which were admitted to Ankara University Medical School Hospital Departments of Pediatrics. The aim of this study is to emphasize that the leg pain might be a symptom of a serious illness. All other pathologic causes should be eliminated while diagnosing the growing pain or musculoskeletal syndrome. First case was a 3-year-old girl who had left leg pain for three months. Physical examination was normal except from the 3/6 systolic murmur. There were pancytopenia in complete blood count and bone marrow aspiration contained remarkable amount of blast cells. Then she was diagnosed as leukemia. The second case was 10-year-old girl who had leg pain continuing for four months. The tuberositas tibia area was sensitive to palpation, piece ossification of the tibial tubercle was detected on the X-ray so she was diagnosed as OsgoodSchlatter disease. The third case was 5-year-old girl who had right leg pain and limping. The X-ray showed a defective structure on the right femoral epiphysis and hence she was diagnosed as Legg-Calves-Pertes disease. The fourth case was 10-year-old boy who suffered leg pain for twenty days. Acute phase reactants were significantly high. X-ray detected lytic lesions on femoral metaphysis. He was diagnosed as acute osteomyelitis. In the fifth case, leg pain started after flu symptoms, creatinine kinase levels were elevated. She was diagnosed as acute viral myositis. In conclusion; leg pain could be a sign of many diseases. Pediatricians should be careful when evaluating the leg pain. Keywords: childhood, leg pain, growing pain - 114 - [P-235] A Rare Agent of Peritoneal Dialysis Related Peritonitis in a Child: Campylobacter Jejuni [P-236] Tuğçe Tural Kara1, Songül Yılmaz2, Halil Özdemir1, Zeynep Birsin Özçakar2, Ahmet Derya Aysev3, Ergin Çiftçi1, Erdal İnce1 1 Department of Pediatric Infectious Diseases, Ankara University Medical School, Ankara, Turkey 2 Department of Pediatric Nephrology, Ankara University Medical School, Ankara, Turkey 3 Microbiology Laboratory, Ankara University Medical School, Ankara, Turkey Peritonitis is a serious problem in children receiving peritoneal dialysis. The overwhelming majority of peritonitis cases are caused by pathogenic bacteria like Staphylococcus epidermidis, Staphylococcus aureus, Streptococcus spp., Enterococcus faecalis and Escherichia coli. Peritoneal fluid culture is important, because unusual microorganisms may be causative. Campylobacter jejuni is a rare peritonitis agent in children. We report a case of C. jejuni peritonitis in a 10-year-old boy who had end stage renal failure due to atypical hemolytic uremic syndrome. He was admitted to hospital with abdominal pain and fever. Peritoneal dialysis fluid was cloudy and microscopic examination showed abundant leukocytes. The diagnoses of peritonitis were established. Then we started intraperitoneal cefepime for empirical treatment. On the 3rd day of treatment clinical and laboratory findings were not exactly recovered. Patient’s temperature did not return to normal range and peritoneal fluid did not become clear. Peritoneal fluid culture revealed C. jejuni which was identificated with Matriks Assisted Laser Desorption/Ionization Time of Flight (MALDITOF) technology, in two different samples. Then oral clarithromycin treatment was added for 10 days. Intraperitoneal cefepime treatment was continued for 14 days. At the end of the therapy, clinical findings recovered, peritoneal fluid culture became negative. In addition, he did not have any peritonitis attacs. In this case, we report a child with C. jejuni peritonitis who was successfully treated. Campylobacter peritonitis is an unusual infection in children, which have been more reported in adult patients in the literature. Oral clarithromycin is more effective for treatment and clinical outcomes are very good. As a result, unusual patogen microorganisms like Campylobacter spp. must be remembered as a causative agent of peritonitis in children who are not responsive to empiric treatment. Keywords: Campylobacter jejuni, continuous ambulatory peritoneal dialysis, peritonitis Petechial-Purpuric Rash and Thrombocytopenia Associated with Parvovirus B19 in a 10-year-old Child Tuğçe Tural Kara, Aysun Yahşi, Tuğba Erat, Halil Özdemir, Ergin Çiftçi, Erdal İnce Department of Pediatric Infectious Diseases, Ankara University Medical School, Ankara, Turkey Parvovirus B19 is a common infection around the world which can cause different skin rashs like maculopapular, erythematous, petechial or purpuric lesions. Fever, artralgia, myalgia and lymphanopathy may also occur. Petechial and/or purpuric lesions may be seen with edema and erythema. Papular-purpuric gloves and socks syndrome and thrombocytopenia are some clinic spectrums of Parvovirus B19. We report a child who was admitted to hospital with petechial rash on her upper and lower extremities. The petechial rash occured especially in her hands and feet, like glove and sock style. Fever was continued during one week. On physical examination bilateral submandibular and cervical lymphadenopathy which were multiple, painful and the largest size 2x1 cm, were localized. In addition aphthous lesions in the mouth, petechiae on the hard palate and gingival hyperemia were revealed. Petechial-purpuric rash occurred on trunk, face and extremities especially on hands and feet. Routine laboratory analysis revealed a hemoglobin level of 12.7 g/dL, white blood cell count of 4700/mm3, platelets count of 125000/mm³. Serologic testing of parvovirus B19 IgM was determined positive and IgG was found negative. Petechial rash began to decline after third day of hospitalization. We believe that the disease is primary Parvovirus infection. The patient was treated with only antipyretic drugs and on follow up the clinical and laboratory findings completely recovered. As a result; Parvovirus B19 can cause some different skin rashs like petechiae and purpura. It should be considered in the differential diagnosis in patients with fever, thrombocytopenia and petechial-purpuric lesions. In healthy children symptomatic treatment is enough to improve the clinical and laboratory signs. Keywords: Parvovirus B19, rash, thrombocytopenia [P-237] Calcaneus Osteomyelitis: Secondary to Guthrie Test Tuğçe Tural Kara1, Tuğba Erat1, Halil Özdemir1, Aysun Yahşi1, Suat Fitöz2, Ergin Çiftçi1, Erdal İnce1 1 Department of Pediatric Infectious Diseases, Ankara University Medical School, Ankara, Turkey 2 Department of Pediatric Radiology, Ankara University Medical School, Ankara, Turkey Calcaneus osteomyelitis is an unusual infection in children. It is seen about 3-10% of all pediatric osteomyelitis. Invasive procedures, prematurity, low birth weight are some causative factors. Clinical signs may be mild. We report a neonate with calcaneous osteomyelitis which was secondary to Guthrie test. In our patient, Guthrie test was made with nozzle on - 115 - the 7th postnatal day. Then three days later swelling occurred on her right heel. On physical examination, 5x5 cm swelling, redness, tenderness and increased temperature was found on her right heel. Patients laboratory test results were as follows; leukocyte count: 16600/mm3, C-reactive protein: 5.1 mg/dl and erythrocyte sedimentation rate: 40 mm/h. X-ray showed osteolytic lesion on the calcaneus which was consistent with osteomyelitis. Superficial tissue ultrasound showed 24x11 mm fluid collection with heavy content was observed on the posterior of the right foot. Extremity magnetic rezonans imaging showed tissue defect, edema-inflammation and contrast enhancement in right foot. All these findings supported osteomyelitis. Puncture and drainage was performed and methicillinsusceptible Staphylococcus aureus was isolated from drainage culture. Then intravenous sulbactam ampicillin treatment was started. S. aureus was detected in nasal swab culture of the patient and her mother. Therefore, topical mupirocin was used during 10 days. Intravenous sulbactam-ampicillin treatment was given for 3 weeks. After discharge, oral amoxicillinclavulanate treatment was received during 3 weeks. At the end of treatment, the lesion was completely resolved and acute phase reactants were normal. In follow-up, the patients recovered without sequelae. As a result, calcaneous osteomyelitis is an uncommon infection in newborns which should be considered in the differential diagnosis of swelling, redness, and increased in temperature on the heel. We should not forget that invasive procedures may cause osteomyelitis. Therefore aseptic technique should be performed before the invasive procedures. Keywords: Calcaneus, Guthrie test, osteomyelitis [P-238] A Case Report of Congenital Lobar Emphysema: A Rare Cause of Hypertension Tuğçe Tural Kara1, Halil Özdemir1, Hüseyin Dindar2, Ergin Çiftçi1, Erdal İnce1 1 Department of Pediatric Infectious Diseases, Ankara University Medical School, Ankara, Turkey 2 Department of Pediatric Surgery, Ankara University Medical School, Ankara, Turkey Congenital lober emphysema is a rare disease which affects especially newborns and infants. It is characterized by pulmoner hyperinflation. Depending on the degree of bronchial obstruction, the clinical presentation is variable like tachypnea, tachycardia and retractions. We report a rare case of congenital lober emphysema in a 38-day-old male infant who admitted to our hospital with tachypnea, tachycardia and retractions. On physical examination he was found to be tachypneic at the rate of 65/min, heart rate of 185/min and blood pressure of 130/70 mmHg (normal range 103/56 mmHg-78/37 mmHg). His oxygen saturation was 85% at room air. In addition, subcostal and intercostal retractions, decreased air intake and bronchospasm were found. Increased aeration of left lung and significant mediastinal shift to the right one were observed on the chest X-ray. Emphysematous changes were seen in the upper lobe of the left lung on the thorax computed tomography which was consistent with congenital lobar emphysema. Then the patient was successfully operated. During the postoperative period, the patient’s blood pressures remained normal. Chest X-ray demonstrated the left lung ventilation decreased and right one shifted at the left side. We thought that due to increased from left to right shift, the renal blood flow reduced by compressing main blood vessels. Reduction in renal blood supply activated the renin angiotensin aldosterone system which caused hypertension by increasing the involvement of water and salt. After left upper lobectomy, the patient’s symptoms significantly decreased furthermore hypertension was not seen again. As a result; congenital lober emphysema is a rare disease which may cause lots of clinical signs like respiratory distress, tachypnea, tachycardia. As well as it can also cause hypertension. The clinicians should think congenital lobar emphysema in the differential diagnosis of respiratory distress and hypertension. Keywords: Congenital lober emphysema, hypertension, respiratory distress [P-239] Bone Marrow Aspiration Complications: Iliopsoas Abscess and Sacroiliac Osteomyelitis Tuğçe Tural Kara1, Halil Özdemir1, Tuğba Erat1, Aysun Yahşi1, Songül Yılmaz2, Ergin Çiftçi1, Suat Fitöz3, Fatoş Yalçınkaya2 1 Department of Pediatric Infectious Diseases, Ankara University Medical School, Ankara, Turkey 2 Department of Pediatric Nephrology, Ankara University Medical School, Ankara, Turkey 3 Department of Pediatric Radiology, Ankara University Medical School, Ankara, Turkey Iliopsoas abscess and sacroliliac osteomyelitis are rare disease in children. History of pelvic trauma may be described in some cases. Bone marrow aspiration may cause some rare complications. We present a boy with iliopsoas abscess and sacroiliac osteomyelitis which are unusual complications after bone marrow aspiration. An 18-month-old boy was admitted to hospital because of fever that was not responsive to antipiretic drugs. Homozygous M694V mutation was identified that confirmed the diagnosis of Familial Mediterranean Fever and oral colchicine was started. However fever continued despite colchicine. Then bone marrow aspiration was performed which was found to be normal. Three months after bone marrow aspiration, he had pain on his left leg. On physical examination, limited movement and pain were found on the left leg. Acute phase reactants were significantly elevated on laboratory examination. Pelvic computed tomography showed left iliopsoas abscess near the muscle with many enlarged lymph nodes and lytic destructive changes in sacrum and iliac bone. Sacroiliac joint magnetic resonance imaging showed T2 signal enhancement near the bone structure and left sacroiliac joint, contrast enhancement areas, cortical thickening and periosteal reaction in the sacroiliac bone. Percutaneous abscess drainage was performed and methicillin sensitive Staphylococcus aureus was isolated from drainage culture. Intravenous ampicillin-sulbactam and intravenous clindamycin were given for 6 weeks. Follow up was excellent; fever disappeared, clinical findings - 116 - improved, left foot pain reduced and he started to stand on his foot. Levels of acute phase reactans decreased. After he was discharged from the hospital, oral amoxicillinclavulanic acid treatment was continued for 2 weeks. On follow up the patient did not have any complaints. As a result; bone marrow aspiration may cause some rare complications. If patients have unusual complaints after aspiration, psoas abscess and sacroiliac osteomyelitis should be considered as a complication. Keywords: Bone marrow aspiration, iliopsoas abscess, sacroiliac osteomyelitis [P-240] Local Complications of Bacille CalmetteGuérin Vaccine at The Injection Site Tuğçe Tural Kara, Halil Özdemir, Tuğba Erat, Aysun Yahşi, Ergin Çiftçi Department of Pediatric Infectious Diseases, Ankara University Medical School, Ankara, Turkey Background&Aims: Bacille Calmette-Guérin (BCG) vaccine can cause some local reactions at the injection site. Although some of these are normal vaccine reactions, unexpected complications may be seen. In literature clinical features, treatment modalities and outcomes of these complications have not been clearly defined. We aimed to determine the incidence of local complications after BCG vaccination, to evaluate the treatment and to compare this result with the literature. Methods: Patients, who admitted to our Pediatric Infectious Diseases Department between January 2010 and June 2015 and developed local complications after BCG vaccination, were retrospectively included in our study. Demographic and clinical informations, laboratory findings, treatment modalities and outcomes were noted from database. Patients who were diagnosed as congenital or acquired immunodeficiency or disseminated BCG infection were excluded from the study. Results: Eight patients are diagnosed as local cutaneous complications after BCG vaccination. The mean age was 7.0±2.23 months. Abscess formation (38.5%), localized inflammation signs like hyperemia, swelling (38.5%), lesion with purulent discharge (12.5%) and eczematous lesions (12.5%) were found at the injection site. Patients were treated with antituberculosis drugs, amoxicillinclavulanic acid, ampicillin-sulbactam and intermittent drainage. In addition two patients were followed without treatment. On follow up all lesions regressed without complications. Conclusions: There is no still consensus about treatment of local complication of BCG vaccination. In our study, some of these complications improved with conservative treatment, but localized abscess formation required drainage. In addition, anti-tuberculosis treatment and antibiotics were given for some indications. As a result treatment of complications should be decided to each patient’s clinical symptoms and underlying disease. Keywords: BCG vaccine, complications, injection site [P-241] Fonsecaea Pedrosoi: A Rare Cause of Maxillary Osteomyelitis and Dental Infection in a Child with Hematological Malignancy Tuğçe Tural Kara1, Halil Özdemir1, Aysun Yahşi1, Tuğba Erat1, Elif İnce2, Ergin Çiftçi1 1 Department of Pediatric Infectious Diseases, Ankara University Medical School, Ankara, Turkey 2 Department of Pediatric Hematology-Oncology,Ankara University Medical School, Ankara, Turkey Fonsecaea pedrosoi is a dermatiaceous fungus which can cause skin and subcutaneous infections. Oral manifestations are commonly seen in children with malignancy. However F. pedrosoi associated dental infection and maxillary osteomyelitis has not been reported in literature. Herein, we report a child with F. pedrosoi infections. A 6-year-old boy with a diagnosis of standart risk precursor B cell acute lymphoblastic leukemia presented with fever on the 43rd day of late intensification chemotherapy. On physical examination black-brown coloured areas were found near the upper left molar tooth with edema of the gingiva. On laboratory evaluation, white blood count was 200/mm3, absolute neutrophil count was 0/ mm3. Paranasal sinus computed tomography showed hyperdense areas and mucosal thickening in the left maxillary sinus and dentin loss with central soft tissue on the upper left second premolar tooth, and osteomyelitis of maxillary bone. Fungal infection was thought to be the cause of these findings. Upon this, tooth was pulled by dentist. Histopathological examination showed fungal hyphae and conidia on the hematoxylin-eosin stained section. Grocott’s Methenamine Silver staining demonstrated the hyphae and conidia of F. pedrosoi. In addition, F. pedrosoi was isolated from the tissue culture. The patient received intravenous liposomal amphotericin B therapy for 5 weeks and he was discharged on oral voriconazole. His complaints decreased markedly. He remains on oral voriconazole therapy because of the possibility of prolonged neutropenia during chemotherapy. Currently, the patient is at the seventh month of treatment without any adverse side-effects. As a result, oral lesions may be seen in patients with ALL in neutropenic period. F. pedrosoi is an unusual causative agent of dental infection and maxiller osteomyelitis. However, it should be considered in the differential diagnosis of black-brown coloured lesions in neutropenic patients with malignancy. Keywords: Dental infection, Fonsecaea pedrosoi, osteomyelitis [P-242] Complete clinical improvement of acute disseminated encephalomyelitis due to Mycoplasma Pneumania infection Ulkem Colak, Emine Tekin, Omer Faruk Aydın Ondokuz Mayis University, Faculty of Medicine, Department of Pediatrics Background&Aims: Mycoplasma pneumania is a common pathogen for respitatory track infections. Mycoplasma infections can affect hematologic, - 117 - cardiovasculary, genitourinary, gastriontestinal and central nervous system. Acute Disseminated Encephalomyelitis (ADEM) can be seen as a central nervous system manifestation of Mycoplasma and seem to have poor prognosis. In this paper we present an eight year old child diagnosed as ADEM assosiated with Mycoplasma Pneumania infection who developed full clinical recovery in three months. Our aim is to show to the clinicians that ADEM due to Mycoplasma Pneumania may have better outcome. Case: An 8 year old boy presented with alteration in consciousness, paraplegia and urinary retention. He had ten days history of fever, cough and general muscle pain. His cerebrospinal fluid (CSF) protein was slightly increased, glucose were normal. MRI showed hyperintense areas at the left capsula interna, right talamus, mesencephalon, pons and left dentate nucleus. The lesions showed increased signal intensity on diffusionweighted images. Acute Disseminated Encephalomyelitis has been diagnosed. The child was treated with i.v. metilprednisolone (Prednol) 30 mg/kg/day for five days and continued with a tappering dose. X-ray of the chest showed bilateral perihilar and peribronchial infiltration, Mycoplasma IgM antibodies were positive at serum so Mycoplasma pneumania infection was diagnosed. The patient was treated with clarithromycin 14 mg/kg/ day for 10 days. He had clinical improvement on day 5, gained normal limb power and developed complete remission in 3 months. Conclusions: To the best of our knowledge this is the first presentation of ADEM case due to Mycoplasma infection recovered fully. We suggest that clinicans can predict a better outcome for the patients who were diagnosed with ADEM due to Mycoplasma Pneumania. Keywords: ADEM, child, mycoplasma [P-243] Central Venous Catheterization Experince in Neonatal Intensive Care Unit Adil Umut Zübarioğlu1, Ali Bülbül1, Ümmühan Öncül1, Pınar Karadeniz1, Taliha Öner2, Evrim Kıray Baş1, Duygu Besnili Acar1, Sinan Uslu1 1 Sisli Hamidiye Etfal Hospital, Neonatal Intnesive Care Unit, Istanbul, Turkey 2 Sisli Hamidiye Etfal Hospital, Pediatric Cardiology Department, Istanbul, Turkey Background&Aims: In this study, we aimed to present our experience about central venous catheterization and evaluate the utility of the central venous catheterization in newborns over last 3 years followed-up at our institution. Methods: The charts of patients were reviewed retrospectively between January 2012 and December 2014. Demographic statistics, catheterizations and laboratory test results investigated for each patient. All of the catheterizations performed by pediatric cardiologist and neonatologist. We present access sites, duration of stay and complications of catheterizations. C-reactive protein (CRP),BUN, creatinin, ALT, AST, Hemoglobin, platelet and white blood cell (WBC) counts were measured before catheterization and after 7 days catheterization. Results: In this time period we performed 30 central vein catheterizations to 23 patients. 27 catheters were implanted successfully. Of the 23 patients, 11 were female (%47,8); they had a mean gestational age of 34,2+4,7 weeks and mean body weight was 3225+1733 grams. Mean catheter implantation day was postnatal 26,2+13,4 days (12-80). Of the 23 patients, 5 (%21,7) were extremely low birth weight infants, 3(%13) were with multiple anomalies, 4(%17,4) were pneumonia and 5(%21,7) werecongenital heart disease. Site of the catheters; 11 (%40,7) right subclavian, 10(%37) left subclavian, 3 (%11,1) left femoral and 3 (%11,1) right femoral veins have been used. In total, 8,3+4,0 (5-18) duration of catheter days were analyzed. There wasn’t significant difference between duration of catheter days and catheter insertion site(p=0.315). There was 2(%7,4) significant catheter related complication; venous thrombosis and pneumothorax. There wasn’t significant difference between catheter complication and catheter insertion site(p=0,947). There wasn’t significant difference between 1st and 7th day of laboratory tests. Conclusions: In this study life threatening complications due to central venous catheterization hasn’t been determined. Therefore this kind of catheterization should be helpful for painful treatments and decreasing workloads. Keywords: central vein catheterization, newborn [P-244] Heterotopic gastric mucosal patch in the distal esophagus – case report Vasile Valeriu Lupu1, Ancuta Ignat1, Gabriela Paduraru1, Angelica Cristina Marin1, Anamaria Ciubara2, Marin Burlea1 1 Pediatrics Department, University of Medicine and Pharmacy “Gr. T. Popa”, Iasi, Romania 2 Psychiatry Department, University of Medicine and Pharmacy “Gr. T. Popa”, Iasi, Romania Heterotopic gastric mucosa (HGM) represents a congenital anomaly consisting of abnormally placed gastric mucosa outside of the stomach. It can be found almost anywhere within the gastrointestinal tract. HGM is most commonly found in the upper esophagus. The diagnosis of HGM is confirmed via endoscopy with biopsy. On endoscopy, HGM is clearly distinct from the esophageal squamous mucosa. HGM located in the distal esophagus needs differentiation from Barrett’s esophagus. Barrett’s esophagus is a well-known premalignant injury for adenocarcinoma of the esophagus. Despite the benign nature of HGM, important complications have been reported. Malignant progression of HGM occurs in a stepwise pattern, following the metaplasia–dysplasia– adenocarcinoma sequence. We present a rare case of a teenage girl with HGM located in the distal esophagus, associated with chronic gastritis and biliary duodenogastric reflux. Endoscopy combined with biopsies is a mandatory method in clinical evaluation of metaplastic and nonmetaplastic changes within HGM of the esophagus. - 118 - Keywords: heterotopic gastric mucosa, teenager, Barrett’s esophagus [P-245] Osteogenesis imperfecta – case report Gabriela Paduraru, Marin Burlea, Ancuta Ignat, Angelica Cristina Marin, Nadia Al Namat, Vasile Valeriu Lupu Pediatrics Department, University of Medicine and Pharmacy “Gr. T. Popa”, Iasi, Romania Osteogenesis imperfecta (OI), a secondary cause of osteoporosis, mainly manifests as bone fragility. It is an inherited disorder of connective tissue integrity; it affects up to one in 10 000 persons. Diagnosis of mild OI is challenging, due to its variable phenotypic expression and inconstant course. Family physicians must maintain a high index of suspicion, as diagnosis, along with proper follow up and counseling, so many complications of this disorder can be avoided. We report the case of an 11-year-old female presented with OI Type IV. From the past medical history, we mention more than twenty fractures, shoulder contusion, proximal bilateral radio-cubital synostosis, dextroconvex dorsal scoliosis, tuberosity of left anterior tibia and pectus excavatum, delay in language development, polymorphic dyslalia, minor right bundle branch block, nocturnal enuresis, psychomotor retardation, mental deficiency, weight loss. OI most often presents after infancy and should be considered, whenever children or adults have recurrent fractures. Early diagnosis of this disease by family physicians will enable initiation of therapy, as well as patient education regarding management of modifiable risk factors linked with osteoporosis (e.g. diet, smoking, alcohol). For many, disease detection can prevent the trauma of separation of parents and children, when OI is misdiagnosed as child abuse. Genetic counseling and family screening could also be offered. The treatment plan should maximize mobility and function, increase peak bone mass and muscle strength, and employ as much exercise and physical activity as possible. Keywords: osteogenesis imperfecta, fractures, child [P-246] A Patient With Atypical Kawasaki Disease: Presented With Arthritis And Hydrops Of Gallbladder Yasemin Ataman, Ayşegül Doğan Demir, Nilüfer Göknar, Dilek Karacanoğlu Bezmialem Vakıf Üniversitesi Tıp Fakültesi Hastanesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı Introduction: Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that primarily affects infants and young children. The diagnosis is based on five of six findings; fever persisting for more than 5 days, conjunctuvitis, stomatitis, indurative dermatitis in the extremities, erythematous rash and lymphadenopathy. The most common complications are coronary arteritis or coronary aneurysm or thrombotic occlusion of the coronary vessels. There are some patients, who do not fulfill the classic diagnostic criteria for KD and have atypical clinical presentations, called atypical (incomplete) KD. In this case we present a patient with atypical KD, presented with arthritis and hydrops of gallbladder. Case: A 3-year-old boy had been referred with complaints of fever, jaundice, pain and swelling in his knees and proximal and distal interphalangeal joints. Physical examination revealed jaundice, bulbar conjunctival injection, erythematous lips and arthritis of the bilateral knees and proximal and distal interphalangeal joints of upper and lower extremities. In laboratory tests, the white blood cell account (WBC), the erythrocyte sedimentation ratio (ESR), serum bilirubin levels and liver function tests were found higher. Abdominal ultrasound showed hydrops of the gallbladder. On the fifth day of the fever, an echocardiogram was performed, coronary artery dilatation was revealed. The patient was diagnosed with KD and intravenous immunoglobulin (2 gr/kg) and aspirin (100 mg/kg/day) were instutied. After IVIG therapy his clinical conditions and laboratory results became better and he was discharged on the 10th day of hospitalization. Discussion: It is important to recognize and treat atypical KD because these patients carry a risk of coronary artery aberrations similar to complete KD. For this reason, physicians should suspect and diagnose patients with KD prematurely, even though they do not meet the complete diagnostic criteria for preventing the potentially life threatening complications of coronary aneurysm. Keywords: Arthritis, Atypical Kawasaki Disease, Hydrops of Gallbladder [P-247] Oksipital lokalized congenital tufted angioma Yasemin Ersözlü1, Ayşe Bozkurt Turhan2, Murat Vural3, Deniz Arık4, Tuğba Barsan Kaya5, Özcan Bör6, Neslihan Tekin5 1 Eskişehir Osmangazi Üniversitesi Tıp Fakültesi Hastanesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı Eskişehir 2 İstanbul Medeniyet Üniversitesi Göztepe Eğitim Araştırma Hastanesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı Çocuk Hematoloji Bilim Dalı istanbul 3 Eskişehir Osmangazi Üniversitesi Tıp Fakültesi Hastanesi Beyin Cerrahisi Anabilim Dalı Eskişehir 4 Eskişehir Osmangazi Üniversitesi Tıp Fakültesi Hastanesi Patoloji Anabilim Dalı Eskişehir 5 Eskişehir Osmangazi Üniversitesi Tıp Fakültesi Hastanesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı Yenidoğan Bilim Dalı Eskişehir 6 Eskişehir Osmangazi Üniversitesi Tıp Fakültesi Hastanesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı Çocuk Hematoloji ve Onkoloji Bilim Dalı Eskişehir Background&Aims: Tufted angioma is a rare cutaneous angiomatous tumor usually occurring between the ages of one and five years, and involving both sexes equally. Congenital onset and diagnosis at birth is a rare condition. In this case report we presented a newborn who had tufted hemangioma located on the occipital region of the head. Diagnosis was confirmed pathologically showing presence of vessels grouped in glomerulus-like patterns - 119 - with the aspect of a “cannon bullet”. Case: A male newborn was delivered at 381/7 weeks with C-section who had tumoral formation localized at the occipital region antenatally. Fetal MRI in 26.GW reported that the tumor was 33x20 mm and hemanjiomatous feature. The baby was followed in the Neonatal Intensive Care Unit for diagnosis and treatment. It was reported as vascular in postnatal doppler USG. Cerebral MRI revealed a hypervascular mass with 4x2x4,5 cm in size. The patient was consulted with Pediatric Neurosurgery and Oncology and operated on postnatal 13th day. The mass was confirmed as tufted angioma by pathological examination. Discussion: Tufted angioma is a benign angiomatous formation may interfere with kaposiform hemangioendothelioma. Both seen in children showing similar immunophenotype and histological examination shows focal glomerular structure and lymphoid infiltrating nodules. Several treatment options are available which has a high recurrence after surgical excision. Regression with systemic corticosteroids, immunosuppressive therapy and subcutaneous interferon alfa have been shown. Follow up is important for tufted hemangioma cases. Keywords: tufted, hemangioma [P-248] RASopaties: two case reports Yasemin Kendir Demirkol1, Zeynep Alp Ünkar2, Hülya Özdemir2, Hülya Selva Bilgen2, Huriye Nursel Elçioğlu1 1 Division of Pediatric Genetics, Derpartment of Pediatrics, Marmara University Medical Faculty, İstanbul, Turkey 2 Division of Neonatology, Derpartment of Pediatrics, Marmara University Medical Faculty, İstanbul, Turkey Introduction: The RASopathies are a relatively common group of phenotypically similar and genetically related autosomal dominant genetic syndromes including Noonan syndrome (NS) and Noonan syndrome with multiple lentigines (NSML, formerly LEOPARD syndrome). NS and NSML can be difficult to differentiate during infancy, but the presence of multiple lentigines, cafe au lait spots, and specific cardiac defects facilitate the diagnosis. Case 1: A 17-day-old male infant was referred for neonatal intensive care unit because of jaundice. The patient was born via caesarian section with 2270gr weight to a 38-year-old gravida 4, parity 4 mother. The parents are not consanguineous but they are from same village. On physical examination his weight was 3220gr (<3rd centile) his length 50cm (-3,-4 SD), and his occipito-frontal diameter 36cm (-3,-4 SD). He had characteristic features of NS along with mild pulmonary valve stenosis (PS) and atrial septal defect (ASD). His karyotype analysis was 46,XY. In exon 13 of the PTPN11 gene p.GLN506Pro(c.1517A>C) a heterozygote mutation was found, classified as NS. Case 2: A 5-month-old female was referred for thromboytopenia, splenomegalia and dismorfic feature. The parents were nonconsanguineous. On physical examination her weight was 4360gr (<3rd centile), height 56cm (-4 SDS), and head circumference 39cm (-2,-3SDS). She had low-set ears, short neck, bilateral mild ptosis, 3/6 systolic murmur, 2cm hepatomegaly and 4cm splenomegaly. Echocardiography revealed ASD and PS. In exon 3 of the PTPN11 gene p.Thr7211e(c.218C>T) heterozygote mutation was found, identified as NS. Discussion: The RASopathies are disorders with an extremely wide spectrum of clinical manifestations and a heterogenous moleculer pathogenesis. Long-term follow up of patients is required to evaluate the evolution of the clinical disease expression and confirm the initial diagnosis. Despite significant advances in moleculer diagnostics, there are still problems with finding the correct clinical diagnosis of the diseases from the group of the RASopathies. Keywords: RASopathies, Noonan Syndrome [P-249] A case of Split Notochord Syndrome: presenting with respiratory failure Yeşim Coşkun1, İpek Akman1, Mustafa Kemal Demir2, Özlem Yapıcıer3, Salih Somuncu4 1 Pediatrics Department, Bahcesehir University School of Medicine, Istanbul, Turkey 2 Radiology Department, Bahcesehir University School of Medicine, Istanbul, Turkey 3 Pathology Department, Bahcesehir University School of Medicine, Istanbul, Turkey 4 Pediatric Surgery Department, Bahcesehir University School of Medicine, Istanbul, Turkey Split notochord syndrome (SNS) is a very rare congenital anomaly. In this report we described a male newborn with a neuroenteric cyst in the posterior mediastinum and multiple vertebrae anomalies presenting with respiratory failure and pulmonary hypertension. A 12 days old full term boy, weighing 2920 gram, was referred to the Neonatal Intensive Care Unit of Bahcesehir University School of Medicine Goztepe Medicalpark Hospital with respiratory failure. At birth, he was hospitalised because of dyspnea and tachypnea. The first diagnosis was congenital pneumonia until he was unable to be weaned from the mechanical ventilation and the cyst in the right lung was seen in a chest computerized tomography. Physical examination showed severe respiratory failure, decreased breath sounds on the right lung. The AP chest radiograph showed a large, well-defined mass in the right hemitorax, displacing the mediastinum to the right, and cervico-thoracic vertebral anomalies. The abdominal magnetic resonance imaging findings showed a homogeneous unilocular high signal cystic mass in the right prevertebral region without extension to the spinal canal. Vancomycin and meropenem were the antibiotic treatment. For the pulmonary hypertension sildenafil was given. He was sent pediatric surgery for the operation of the cyst in his right lung. The mass was in the posterior mediastinum and it was removed totally. The histopathological finding revealed as Neuroenteric cyst. He was discharged from the hospital at 53 days of age. Since SNS is seen very rarely, the management of SNS - 120 - must be tailored on the different anomalies present in each case. In the present case excision of cyst resulted in improvement of respiratory symptoms. Early diagnosis and treatment improves prognosis. Keywords: split notochord syndrome, neuroenteric cyst, respiratory failure [P-250] A rare cause of vomiting: Annular pancreas Yasin Şahin1, Yusuf Can Doğan2, Hasret Ayyıldız Cıvan1, Şenol Emre3, Osman Uzunlu3, Gonca Topuzlu Tekant3, Hatice Arıöz Habibi4, İbrahim Adaletli4, Fügen Çullu Çokuğraş1, Tufan Kutlu1, Tülay Erkan1 1 Istanbul University Cerrahpasa Faculty of Medicine, Depatment of Pediatrics, Division of Gastroenterology, Istanbul, Turkey 2 Istanbul University Cerrahpasa Faculty of Medicine, Depatment of Pediatrics, Istanbul, Turkey 3 Istanbul University Cerrahpasa Faculty of Medicine, Depatment of Pediatric Surgery, Istanbul, Turkey 4 Istanbul University Cerrahpasa Faculty of Medicine, Division of Pediatric Radiology, Istanbul, Turkey Background & Aim: Anular pancreas is a rare congenital anomaly which consists of a ring of pancreatic tisue partially or completely encircling the second part of duodenum. It may affect anyone from the neonatal period to the adult. It is difficult to diagnose because it can present in a wide range of clinical conditions. Although the cases are also reported in adults, the symptomatic cases often refer in infancy or early childhood. Here, we report a case of duodenal obstruction associated with anular pancreas, which has rarely been reported in late adolescent period. Case: The 17 years female patient has had a non- bilious vomiting accompanied with abdominal pain especially 5-10 minutes after meals for seven years. She has lost 7.5 kg in last 2 months. Esophagogastroduodenoscopy was performed for etiological evaluation. The endoscopic appearance of upper esophagus was dirty yellow color and biopsy was taken. The microbiological analysis of the tissue example showed candida infection. The distal part of duodenum after bulbus could not be visualised, referring to obstruction. The contrast X-ray imaging of the upper gastrointestinal tract showed no passage of contrast material through the bulbus. These findings of obstruction were aproved by contrast-enhanced CT-scan. The diagnosis of annular pancreas was considered. Then, this diagnosis was approved by operation. ’’Diamond shaped duodeno-duodenostomy’’ was carried out. Conclusion: Annular pancreas which may be seen at any age by taking into account, should be considered in the diagnosis of patients with non-bilious vomiting particularly after meals for a long time. Keywords: vomiting, annular pancreas, child [P-251] Raoultella terrigena, a rare pathogen in neonatal intensive care unit Zeynep Alp Ünkar1, Gülşen Akkoç2, Ebru Eren3, Hülya Özdemir1, Aslı Memişoğlu1, Nurver Toprak3, Ahmet Soysal2, Hülya Selva Bilgen1, Eren Özek1 1 Marmara University Faculty of Medicine, Department of Pediatrics, Division of Neonatology 2 Marmara University Faculty of Medicine, Department of Pediatrics, Division of Pediatric Infectious Diseases 3 Marmara University Faculty of Medicine, Department of Microbiology Raoultella terrigena (formerly Klebsiella terrigena) is a gram-negative, oxidase-negative bacillus, belonging to the family Enterobacteriaceae. First isolated in 1981 from soil and water, it’s a rare pathogen in human hosts. There are only two adult cases in the literature – one endocarditis and one sepsis – reported so far and a congress abstract of neonatal sepsis in the neonatal intensive care unit (NICU) in Gaza, reporting its incidence as 25.5% among Klebsiella species. We present the first neonatal case of R. terrigena sepsis from Turkey. The first-born of thirty gestational-week-old dizygotic twin boys was intubated and given surfactant in the delivery room. He was transferred to NICU where umbilical venous catheter was inserted and empiric ampicillin gentamycin therapy was started. He was extubated in 18 hours and didn’t need any form of assisted ventilation later on. Enteral feeding was initiated on the 2nd day of life (DOL). On DOL 8 he presented with increased gastric residuals, hyperglycemia, cutis marmoratus and apnea attacks for which he was intubated. Sepsis screening was positive with increased C-reactive protein (CRP) and procalcitonin (PCT) levels. Cerebrospinal fluid (CSF) analysis was normal. Blood, urine and CSF cultures were taken. Considering the unit’s flora, empirical vancomycin and meropenem were started. Blood culture revealed R. terrigena resistant only to ampicillin and sensitive to amoxicillin-clavulanate, gentamycin, cefuroxime, cefuroxime-axetil and trimethoprim-sulfamethoxazol. Meropenem was switched to cefepime. CRP and PCT decreased gradually and the baby improved clinically. Patient was extubated on the third day of antibiotics and whole therapy was completed to 21 days. Clinical significance and pathogenesis of R. terrigena is still uncertain. Despite occurrence in human clinical samples and colonization of feeding tubes, there are few case reports about human infections. Considering that feeding tubes are commonly used in NICU, attention should be paid to this seemingly-rare gram-negative pathogen. Keywords: Raoultella terrigena, neonatal sepsis, NICU - 121 - [P-252] Double aneuploidy: Down - Klinefelter Syndrome Zeynep Alp Ünkar1, Yasemin Kendir Demirkol2, Aslı Memişoğlu1, Huriye Nursel Elçioğlu2, Eren Özek1 1 Division of Neonatology, Derpartment of Pediatrics, Marmara University Medical Faculty, İstanbul, Turkey 2 Division of Pediatric Genetics, Derpartment of Pediatrics, Marmara University Medical Faculty, İstanbul, Turkey Background: Trisomy 21, also named Down syndrome (DS), is caused by the presence of an additional autosome, affecting 1/700 live births. Klinefelter Syndrome (KS) is a genetic condition in which humans have an extra X chromosome, resulting in a 47,XXY karyotype. Double Aneuploidy involving both autosomal and sex chromosomes is rarely described. Presence of coincidental two meiotic non-disjunctional events, can lead to double aneuploidy which called as trisomy of two different chromosomes. Case: A 3-month-old male, born to non-consanguineous parents referred to our polyclinic because of dysmorphic features. Mother’s age was 30 years, and father age was 28. Physical examination showed flat occiput, hypertelorism, low-set ears, high narrow palate and hypotonia. Chromosomal analysis showed a karyotype 48, XXY+21, a double aneuploidy of DS and KS. Conclusion: In conclusion, Down-Klinefelter syndrome is a rare occurrence. It presents trisomy 21 characteristics early in life and klinefelter syndrome features after 10 months of age. Keywords: Down Syndrome, Klinefelter Syndrome, double aneuploidy [P-253] Can Mycoplasma pneumoniae be a trigger for Type 1 Diabetes? Zeynep Hızlı1, Zehra Yavaş Abalı2, Hacer Aktürk3, Ahmet Uçar2, Şükran Poyrazoğlu2, Firdevs Baş2, Ali Ağaçfidan4, Rüveyde Bundak2, Feyza Darendeliler2, Ayper Somer3 1 Istanbul University Faculty of Medicine, Istanbul, Turkey 2 Department of Endocrinology, Istanbul University Faculty of Medicine, Istanbul, Turkey 3 Department of Infection, Istanbul University Faculty of Medicine, Istanbul, Turkey 4 Department of Microbiology, Istanbul University Faculty of Medicine, Istanbul, Turkey Background: Type 1 diabetes mellitus (T1DM) is a multi-factorial disease. One of the possible theory for disease is various viruses do play a role in the pathogenesis in individuals with genetic susceptibility. Other environmental factors were reported in many studies. Bacterial pathogens also suggested as a trigger. In this report, we presented a child with T1DM presenting with Mycoplasma pneumoniae infection. Case: A 10-year-old boy was referred by an otolaryngologist because of weight loss, after diagnosis of right acute otitis media and left tympanic membrane perforation. He had polydipsia, polyuria and enuresis nocturne. His height was 135.5 cm (-0.20 SDS), weight 40.6 kg (1.33 SDS) and body mass index 22.2 kg/m2 (1.68 SDS). He had tachycardia (125/min), tachypnea (36/min) and his blood pressure was 95/60 mmHg. He had no fever. He had hyperglycemia (408 mg/dL), ketonuria and metabolic acidosis (pH: 7.09 HCO3: 5.1 mmol/L). Basal insulin and C-peptide levels were 2.2 μU/mL and 0.3 ng/mL, respectively. HbA1c was 12.8%. Ketoacidosis was corrected in 18 hours and subsequently treated with subcutaneous insulin. Specific serum tests for diabetes; glutamic acid decarboxylase antibody – GADA 26.9 U/mL (<1.0), islet antibody-IA 6.13 U/ mL (<1.0) and HLA- DRB1-DR3&DR4 genotypes were positive. At the same time his nasopharyngeal swab for M. pneumoniae DNA was detected positive by polymerase chain reaction (PCR). Also positive M. pneumoniae IgM and IgG antibodies identified by enzyme-linked immune assay (EIA). These findings suggest that acute otitis media caused by M.pneumoniae which is a rare pathogen for this infection, triggered diabetic ketoasidosis. Conclusion: As far as we know, this is the first report of an association between DKA and M. pneumoniae infection. We suggest that infection should be kept in mind developing of T1DM. Further study is needed to elucidate the relationship between T1DM and M. pneumoniae infection. Keywords: M. pneumoniae, Type 1 diabetes [P-254] Results of the Hearing Screening Tests of Newborn Children for 3 years Adil Umut Zübarioğlu, Sinan Uslu, Zeynep Işcan, Ali Bülbül, Duygu Besnili Acar, Evrim Kıray Baş Department of Neonatology, Sisli Hamidiye Etfal Education and Research Hospital, Istanbul, Turkey Background&Aims: To share data which showed up at the end of the hearing screening tests on newborn children at our hospital. Methods: The work was carried out on 4438 newborn children, who were born at our hospital and near hospitals, at Şişli Hamidiye Etfal Hospital between July 2012 and July 2015. The newborns who were not responsive to unilateral and bilateral emissions were invited to control for 15 days later. At first control, newborns who passed TEOAE(Transient Evoked Otoacoustic Emission) test for both sides of ears are determined as they passed the hearing test. The newborns who failed TEOAE test at second control were called for third control and performed ABR(Auditory Brainstem Response) on them. Results: TEOAE test was performed for 8876 newborn children which was the 91 percent of all 9754 newborns. 969 (10.9%) newborns who failed the test were called for checking. 311 newborn (3.2%) of recalled ones failed the second test and they were called for the ABR test. At the end of the ABR test, 71(0.8%) newborns were dispatched on the suspicion of hearing loss. It’s important to say that 52(0.6%) newborns are detected as patients of unilateral hearing loss and 19(0.2%) newborns are detected as patients of bilateral hearing loss. - 122 - Conclusions: The hearing screening tests of newborn children are noninvasive and quick tests. Thanks to these tests, cases of hearing loss which can be detected at at least 2-3 ages of children, can be determinated and started treatment at first 3 months of newborns. However, these tests must become widespread and must be performed on all newborn children. Keywords: hearing loss, screening [P-255] Transient Pseudohypoaldestronism in an infant with urinary tract infection associated with urinary tract malformation Zeynep Karakaya, Gamze Ozgurhan, Didem Ocal Topcu, Bulent Hacihamdioglu Suleymaniye Maternity and Children’s Training and Research Hospital-Department of Pediatrics -Istanbul, Turkey Introduction: Uncommon presentation of hyperkalemia,hyponatremia and dehydration may be life threatening in infancy and therefore need immediate investigation to found out possible causes particularly congenital adrenal hyperplasia (CAH) and other causes of hypoaldestronism. Transient pseudo-hypoaldosteronism (PHA) is a rare condition characterized by renal resistance to the action of aldosterone; patients exhibit salt wasting and hyperkalemia associated with high levels of plasma aldosterone. Here we report a case with transient PHA caused by urinary tract obstruction associated with urinary tract infection (UTI). Case Report: Patient was 4-month-old previously healthy male,with uncomplicated birth history. There was a history of hydronephrosis, which was detected on antenatal screening. He was admitted to our pediatric emergency department with complaints of fever, poor sucking and restlessness. Physical examination revealed moderate dehydration and fever with 38.5oC, other systemic examinations were unremarkable. Initial blood samples revealed hyponatremia with sodium level 122 mmol/L and hyperkalemia with potassium level 6.6 mmol/L. Urinalysis revealed pyuria with nitrite and urine culture was positive for Klebsiella pneumonia.Renal sonography demonstrated bilateral hydronephrosis with megaureters. Serum sodium and potassium level normalized within 24 hour after intravenous fluids and antibiotic therapy. The diagnosis of pseudohypoaldosteronism became apparent when serum aldosterone level obtained on admission was markedly elevated to 664 ng/dl (reference range 3.7-43). Discussion: Transient PHA is a condition that presents with hyponatremia and hyperkalaemia. The initial biochemical picture is similar to adrenal hypoplasia, CAH or secondary PHA. UTI with or without any underlying malformation may also cause transient renal tubular resistance to aldosterone, manifested by low sodium and high potassium level. This condition should be kept in mind when infants present with salt wasting or hyperkalemia. Urinalysis and renal ultrasonography should always be performed in order to allow early recognition of transient PHA to avoid unnecessary treatment. Keywords: Transient Pseudohypoaldestronism, Urinary Tract Infection [P-256] A child with methamphetamine Intoxication Asli Kibris1, Zeynep Karakaya1, Gamze Ozgurhan1, Oznur Vermezoglu1, Suray Aliskan2 1 Suleymaniye Maternity and Children’s Training and Research Hospital,Department of Pediatrics,Istanbul Turkey 2 Beylikduzu Medilife Hospital, Pediatric ICU, Istanbul Turkey Introduction: Acute altered level of consciousness (LOC) is the common presenting complaint to pediatric emergency department.It is usually emerged by toxic, metabolic and structural causes. Methamphetamine being a rare cause of acute altered LOC in intoxicated cases,may cause hypertension, tachycardia, severe agitation and psychosis. It is a synthetic psycho stimulant and hallucinogenic agent that can be easily manufactured with low cost via readily available chemicals at meth labs prepared at home.Here we report a child evaluated by altered LOC and diagnosed as methamphetamine Intoxication. Case Report: 26-month-old female child, admitted to pediatric emergency department with presenting complaints of acute onset of lethargy and agitation.Past medical history of patient was unremarkable.On physical examination;patient was lethargic and pupils were dilated. Her blood pressure was 95/59 mmHg, heart rate was 140/min, capillary filling time was prolonged and axillary temperature was 36.5 C. Other systemic examinations were unremarkable. Urine toxicology screen was positive for methamphetamine and she was diagnosed as methamphetamine intoxication. Other laboratory tests were found insignificant and cranial BT was found normal. She was followed in pediatric intensive care unit as intubated because of apneic breathing and then discharged after three days with full recovery. Conclusion: With the growing prevalence of methamphetamine use and production in home laboratories, children are at high risk of injuries resulting from living in a drug-endangered environment. We present a case of a child harmed by the production of methamphetamine at home. Acute alteration of LOC in otherwise known healthy patients,without history of previous trauma, belonging conflicting and contradictory parents are subjected to be investigated for intoxication. Although methamphetamine intoxication is rare but should be kept in mind by pediatricians. Keywords: Methamphetamine Intoxication, Stimulant Toxicty in Children - 123 - [P-257] And thrombus formation in dilated atrium is also a rare complication. Herein we present a patient with restrictive cardiomyopathy. A patient with pseudo vitamin D-deficiency rickets Gamze Ozgurhan, Zeynep Karakaya, Pınar Selimoglu, Bulent Hacıhamdioglu Suleymaniye Maternity and Children’s Training and Research Hospital -Department of Pediatrics-Istanbul Turkey Pseudo vitamin D-deficiency rickets (PDDR) (Vitamin D-dependent rickets Type I) is an autosomal recessive disorder characterized by hypocalcemia, rickets and low or undetectable level of serum 1, 25-dihydroxyvitamin D [1, 25(OH) 2D3]. This disorder results from a defect in the renal 25-hydroxyvitamin D 1α-hydroxylase, the key enzyme in the pathway of vitamin D metabolism. We reported an infant who diagnosed as PDDR. The 15-month-old female patient presented to our pediatric endocrinology department with inability to walk. She used adequate amount of vitamin D3; 400 IU/day till 1-years-of age. There was a history of using total 600.000 units of oral vitamin D3 just after 1 years of age due to difficulty in inability to walk. She is the child of first-cousin marriage, with no known family history of metabolic bone disease. Physical examination revealed short stature, frontal bossing, mildly reduced muscle tone, normal teeth development and widening of the wrist. There was no rachitic rosary, Harrison’s sulcus or prominent bone deformity. There was no evidence of latent tetany. Initial investigations resulted as: low serum calcium (5.9 mg/dl, normal 8.5-10.5) and phosphorus (3.4 mg/dl, normal 4.2-6.4), high serum parathyroid hormone (PTH) (925 pg/ml, normal 15-65), high serum alkaline phosphatase level (2531 IU/l, normal 124-332 U/L), a normal serum 25OHD3 (41 ng/ml), and very low level serum 1,25(OH)2D3 (<3.67 ng/ml, normal 25-153 ng/ml) suggesting 1α-hydroxylation defect. Radiological workup showed findings of generalized fraying and cupping at the metaphysis. Calcitriol and oral calcium treatment was initiated with a clinical diagnosis of PDDR. Children with inability to walk should be investigated for rickets by pediatricians. PDDR usually presents with rachitic changes nonresponsive to Vitamin D3 treatment with low circulating levels of 1, 25-Dihydroxyvitamin D3. It must be distinguished from nutritional rickets and vitamin D-dependent rickets Type II in order to plan appropriate therapy. Keywords: Pseudo vitamin D-deficiency rickets, Vitamin D-dependent rickets Type I [P-258] A Rare Complication of Restrictive Cardiomyopathy in Pediatric Age Group Sertac Hanedan Onan, Ozgul Yigit, Zubeyde Kilinc Yaprak, Sultan Bent, Leyla Besel, Erkut Ozturk, Alper Guzeltas Bagcilar Training and Research Hospital, Pediatric Department, Istanbul, Turkey Background&Aims: Idiopathic restrictive cardiomyopathy is a rare disease in children and is characterized by ventricular diastolic dysfunction with dilated atria and normal ventricular size and thickness. Case: A 39 months-old Syrian refugee girl, with previous history of restrictive cardiomyopathy and three siblings’ death by heart disease, presented to the emergency room complaints with swelling of legs and difficulty to breath. On physical examination, she appeared orthopneic and dispneic. BPM: 45/min, HR:150/dk, BP: 105/55 mmHg. There was 3/6 pansystolic murmur on the apex and left lower sternal border. She had a distanded abdomen and a massive hepatomegaly with liver edge 8 cm below the right costal margin. Her legs are cold and pitting edema was determined. In telecardiography cardiothoracic index was increased. ECG showed sinus tachicardia, low voltage, P-mitrale and P-pulmunale. In Echocardography biventricular restriction,biatrial dilatation, severe mitral and tricuspid regurgitation were demonstrated. There was also a giant solid mass detected in the anterior side of right atrium. Fractional shortening was%39. In laboratory studies hematocrit and leucocyte count were normal, platelet count:134.000/mm3 fibrinogen:117mg/ dl(normal values:150-400mg/dl), D-dimer: 9,60ug/ L(normal values:0-0.5ug/L). These findings suggested right atrial thrombus and low moleculer weight heparin therapy was started. She has been using furosemide, captopril and spironolactone for fifteen months till the first diagnosis. And there was no history of dysrhythmia. Anticoagulant therapy was not used because of difficulties in close monitorization. Conclusions: We report this case to discuss the management of atrial thrombus formation in restrictive cardiomyopathy and emphasize the thrombus formation can be seen in pediatric age group even during sinus rythym. Keywords: Restrictive Cardiomyopathy, thrombus [P-259] Vitamin D deficiency in newborns and mothers Abdullah Sert, Ozgul Yigit, Gamze Yildirim, Meltem Erol, Ozlem Bostan Gayret, Zubeyde Kilinc Yaprak Bagcilar Training and Research Hospital, Pediatric Department, Istanbul, Turkey Background&Aims: Vitamin D status at birth and during early infancy, when breast milk is the predominant source of nutrition, is determined by maternal vitamin D status. The aim of the study is to determine the parameters effecting the maternal vitamin D levels and the correlation between maternal and newborns’ vitamin D levels. Methods: Between January 2015 and March 2015, 108 neonates, who were born in Bağcılar Training and Resourch Hospital obstetrics clinic, and their mothers were included to the study. We questioned the women about dressing style(covered vs uncovered), exposure to sunlight, vitamin supplements usage and consumption of fish and milk products. And blood samples for Vitamin D levels were obtained both women and their babies. The levels of 25-(OH) D were classified in four groups (< - 124 - 5ng/mL, 5-10ng/mL, 10-15ng/mL, >15ng/mL). Results: We include 108 mother and newborn couple to study. de women were dressed covered (%94), exposure to sunlight was less than 2 hour in %88 of the women. The usage of vitamin D supplements was %64 (n=69) and the consumption of fish and milk pruducts were %76, %87 respectively. The 25-(OH)D levels was <5ng/mL in %81 of women, and %75 of newborns. Only significant correlation determined between mother and newborn 25-(OH)D levels(p<0.05) Conclusions: Newborn’s vitamin d levels reflects their mother’s levels. Adequate vitamin d stores in women would prevent diseases caused by vitamin d deficiency in early infancy. Keywords: Vitamin d, mother, infant [P-260] Gastritis in Childhood Ancuta Ignat Pediatrics Department, University of Medicine and Pharmacy “Gr. T. Popa”, Iasi, Romania Gastritis is acute or chronic, diffuse or focal inflammation of gastric mucosa. Gastroduodenal ulcer, as well as gastritis is a multifactorial disease characterized by limited loss of substance to the mucosa, submucosa and muscle caused by imbalance of mucosal protection mechanisms and aggressive factors. Bacterial infection, nonsteroidal anti-inflammatory drugs, bile reflux, autoimmunity or allergic response are pathogenic links in children with gastroduodenal diseases. H. pylori gastritis is a primary infection of the stomach and is the most frequent cause of chronic gastritis. Chronic reactive chemical gastritis is associated with long-term intake of aspirin or NSAIDs. Noninfectious diseases are the usual cause of gastric granulomas; they include Crohn disease, sarcoidosis, and isolated granulomatous gastritis. Eosinophilic gastritis can be part of the spectrum of eosinophilic gastroenteritis. Radiation gastritis usually occurs 2-9 months after initial radiotherapy. Ischemic gastritis is believed to result from atherosclerotic thrombi arising from the celiac and superior mesenteric arteries. Clinical data, especially the increasing quality and accuracy of paraclinical diagnosis, result in a low rate of complications of these disorders, through the use of constantly improved multimodal treatment strategies. loss of weight and stature. Also, H. pylori is involved in diabetic patients’ status and food allergies. Children differ from adults with respect to H. pylori infection in terms of the prevalence of the infection, the complication rate, the near-absence of gastric malignancies, age-specific problems with diagnostic tests and drugs, and a higher rate of antibiotic resistance. Increasing the quality and accuracy of detection of infection with H. pylori causes a low rate of complications of these diseases by applying constantly improved treatment strategies. Keywords: Helicobacter pylori, digestive disease, extradigestive diseases, child [P-262] Particularities of the Gastroesophageal Reflux Disease in Childhood Vasile Valeriu Lupu Pediatrics Department, University of Medicine and Pharmacy “Gr. T. Popa”, Iasi, Romania Gastroesophageal reflux (GERD) is a frequent entity in the pediatrics that is still underdiagnosed (“the third millennium’s disease”). The gastroesophageal reflux disease represents the intermittent or permanent passing of the stomach content into the esophagus, which triggers a whole set of digestive, respiratory and/or neurobehavioral symptoms or the absence of any symptoms. GERD occurs in approximately 1 in 300 infants. Clinical polymorphic symptoms are not only dominated by post-food vomiting, but, in many cases, by respiratory symptoms (night coughing at episodes of apnea, recurrent wheezing, aspiration pneumonia and even sudden death syndrome). Frequent, effortless regurgitation of feeds is common during early infancy. GERD has triple therapy: general measures, surgical and pharmacological therapy. The initial drugs (prokinetics agents) were replaced in the last period with proton pump inhibitors (PPI), because they are able to cause the disappearance of the symptoms of reflux in most cases. Key words: gastroesophageal reflux, respiratory manifestations, child [P-263] The demographic features and treatment complications of acute leukemia patients followed by hematology clinic between 2004-2008 Adem Karbuz1, Neşe Yaralı2, Pamir Işık2, Ali Bay3, Abdurrahman Kara2, Bahattin Tunç2 1 Pediatric Infectious Disease, Okmeydanı Education and Research Hospital, İstanbul, Turkey 2 Ankara Children’s Hematology Oncology Training and Research Hospital, Ankara, Turkey 3 Gaziantep University Faculty of Medicine Hospital, Gaziantep, Turkey Key words: gastritis, gastroduodenal ulcer, child [P-261] Helicobacter Pylori Infection in Children Angelica Cristina Marin Pediatrics Department, University of Medicine and Pharmacy “Gr. T. Popa”, Iasi, Romania Helicobacter pylori (H. pylori) is a gram-negative bacillus responsible for one of the most common infections found in humans worldwide. The prevalence is high in developing countries and the infection is acquired at a young age. Infection with H. pylori is a cofactor in the development of three important upper gastrointestinal diseases: duodenal or gastric ulcers, gastric cancer, and gastric mucosa-associated lymphoid-tissue (MALT) lymphoma. Recent research argues and the involvement of H. pylori in the occurrence of extradigestive diseases. Chronic infection with H. pylori is associated with anemia, ABSTRACT Aim: Our aim was to analyze the patients diagnosed with acute leukemia and received chemotherapy in our hospital during five years, retrospectively. Their epidemiological, clinical and laboratory features were determined. Also, all the systemic complications of these patients were reviewed in order to contribute to therapeutic approaches of such patients. Methods: A total of 97 patients who were followed by the pediatric hematology department between - 125 - January 2004 and December 2008, were recruited to our study. Their demographic, clinical, laboratory, radiological and echocardiographic characteristics were documented. Finally, 94 of these patients could be evaluated in terms of all their systemic complications. Results: The mean age of cases was 71.9 + 5 months and the age interval was 2-201 months. 65% of the cases were male and 35% of them were female. The diagnoses were acute lymphoblastic leukemia in 84.5% and acute myeloid leukemia in % 15.5 of the patients. While the most common manifestations were fever (63.9%) and fatigue (57.7%), the most common physical examination findings were hepatomegaly (77.3%) and pallor (68%) at the diagnosis. The complications observed during chemotherapy in the patients were at least one episode of febrile neutropenic attack in 90%, five-fold increase in hepatic enzymes in 80.8%, mucositis in 43.6%, allergy to l-asparaganiase in 28.7%, and compliance problem in 9.5%. Conclusions: The childhood leukemia has a potential to cause complications in many organ systems due to the both disease itself and chemotherapy medications used for the treatment. Multidisciplinary management and follow up are prerequisites. [P-263] Collectıng And Observıng The Growths Of The Cases Wıth Cleft Lıp And Cleft Palate Avidan Kızılelma Yiğit1, Şerife Suna Oğuz2 1 Division of Neonatology, Okmeydanı Education and Research Hospital, Istanbul, Turkey. 2 Division of Neonatology, Zekai Tahir Burak Maternity Teaching Hospital, Ankara, Turkey. Objective: Cleft lips and palates are frequent congenital malformations of the head-neck area. There are no sufficient data on its frequency in our country. The purpose of this study is determining the incidence of cleft lips and palates in the infants born in Zekai Tahir Burak Maternity Teaching Hospital and evaluating the growth results of these patients. Material and Methods: The infants born in Zekai Tahir Burak Maternity Teaching Hospital in 2010 diagnosed with cleft lips and/or palates in physical examinations by the pediatrist were included in the study. By considering the number of the infants born in the hospital in the same year, an approximate incidence was determined. All of the patients were provided with prosthetic nutrition plates, which were prepared specially for the patients. They were discharged to be followed in the relevant departments. The body heights and weights of the patients who could be reached at the age of four were recorded and their growth were evaluated. Results: 17990 live births were observed during the study; and in 19 cases, cleft lips and palates were determined. The frequency of the disease was found as 1 infant in every 947 live births. 9 patients could be reached at the age of 4. Their body heights and weights were recorded. It was observed according to the percentile curves formed by Olcay Neyzi et al. that the body height and weight of 1 patient was below 3 percentile, and the body heights and weights of the other 8 patients were below 3-50 percentile. Conclusion: The frequency of cleft lips and palates in Zekai Tahir Burak Maternity Teaching Hospital is in accordance with the literature. The proper treatment for these frequent malformations in the right time is necessary for a normal growth. [P-264] Successful management of a complicated orbital cellulitis with ampicillin-sulbactam monotherapy Bilge Aldemir-Kocabaş1, Bülent Çekiç2, İclal Erdem Toslak2 1 Pediatric Infectious Disease, Antalya Education and Research Hospital, Antalya, Turkey 2 Clinic of Radiology, Antalya Education and Research Hospital, Antalya, Turkey Orbital infections require prompt diagnosis and treatment because of the risk of severe complications such as subperiosteal and epidural abscesses. Orbital cellulitis is an infection that concerns soft tissue behind the orbital septum. Ampicillin-sulbactam is an appropriate treatment for etiologic agents such as S. aureus, S. pyogenes, H. influenzae, S. pneumoniae and anaerobs responsible from orbital cellulitis. An 8.5 years old girl was hospitalized at an outpatient clinic for 5 days with complaint of high fever. Subsequently, she was referred to our clinic wherefore proptosis, swelling, erythema and warmth of left eyelid occurred. The eyelid was completely closed. There was no limitation of eye movements or findings of ophtalmoplegia at presentation. Laboratory findings were as following: Hemoglobin: 9,9 g/dL, leukocyte: 22,800/mm3, platelets: 890,000/ mm3, sedimentation rate: 118 mm/h, CRP: 28,8 mg/L. Ampicillin-sulbactam (200 mg/kg/day) and ibuprofen (10 mg/kg/dose, q8hr) were initiated for treatment of orbital cellulitis. Additionally, orbital CT revealed a subperiosteal abscess (22X14 mm in size) and epidural abscess. At the third day of treatment proptosis was started to resolve and the acute phase reactants regressed to normal ranges at the end of first week. The duration of treatment completed to eight weeks because of epidural involvement. Magnetic resonance imaging revealed a regression of the subperiosteal abscess enlargement to 4x2 mm in size and disappearing of the epidural abscess. She discharged from the hospital with oral amoxicillinclavulonate for four weeks. All complications were fully recovered at the end of 12th of the treatment. Effective monoterapy is very important for both drug adverse effects and convenience of use. So, if clinical, laboratory findings and fever resolve with monoterapy it is not necessary to change the antibiotic regimen. Key Words: Orbital cellulitis, epidural abscess, ampicillin-sulbactam [P-265] Wilson Disease Dr. Kyaw Zin LATT and Dr. Zeynep GÖR Introduction: Wilson disease is an autosomal recessive disorder which is related to the copper metabolism and characterized by degenerative changes in the liver, brain, cornea and the other tissues. Presented is a 3 year old male patient who admitted to the hospital due to the icterus of the cornea and skin, who was evaluated for jaundice and whose serum ceruloplasmin level was found to be lower than normal. Case Presentation: A 3-year old male patient was admitted to the hospital due to the icteric appearance which started about a week ago. The liver function tests were found to be elevated (AST: 2949 IU/L ALT: 1762 IU/L ALP 190 IU/L GGT 52 IU/L). The serum total - 126 - bilirubin level is 11.63 mg/dL and the direct bilirubin level was 9.9 mg/dL. The PT was slightly prolonged (16.59 sec.) and INR was 1.46. His peripheric blood film showed no signs of hemolysis. The abdomen USG revealed hepatomegaly; liver (134 mm) and the spleen (106.5 mm). The viral hepatitis markers were all negative. The hepatic portal Doppler USG showed no abnormality. IV contrasted Abdominal CT revealed slight elevated liver size (154 mm) and generalized oedema in the periportal area of the liver. The spleen was slightly enlarged (102 mm). The alpha 1 antitrypsin level was 170 U/L, excluding alpha 1 antitrypsin deficiency. Autoimmune hepatitis markers such as ANA, ASMA and LKM antibody levels were all negative. Celiac disease antibodies levels were also negative. Ophthalmologic examination showed no abnormal findings. The serum ceruloplasmin level was 15.6mg/dL in favour of the diagnosis of Wilson Disease. Copper level in the 24 hour urine collection was 163.5mcg/g (high). Liver biopsy was performed and the hepatic copper content was 586 ug/g (normally <10 ug/g dry weight). Discussion: Wilson Disease is one of the causes of hyperbilirubinemia with the incidence of 1/ 100.000 to 1/500.000 birth. However It should be always kept in the physician’s minds when evaluating a patient with hyperbilirubinemia, together with hematologic pathologies, autoimmune and viral hepatitis and the other causes, considering the possibility that the disease progress can be controlled by the right diagnosis and treatment. [P-266] A case report: Progressive familial intrahepatic cholestasis type 2 with liver transplantation Introduction: Progressive familial intrahepatik cholestasis (PFIC) type 2 was previosly known as Beyler’s syndrome (1) and is a resault of muatation in the ABCB 11(ATP binding cassette (ABC) familiy B,member 11) encoding BSEP ,located on chromosome 2(2q24)(2).BSEP is transporter protein,expressed at the canalicular membrane of hepatocyte (3).İt is the main exporter of bile acids from hepatocyte of to caniluli against a concertration gradient.Early recognition of PFIC -2 which can had to hepatic failure in generally frist decade contribute to the therapy of discase.Our purpose is to present a PFIC case in which hepatic transplantation was needid because of hepatic failure devoleped in early period. 5 month control *ALT: 121 IU *AST; 184 IU *T.PTROTEİN: 5.0 MG/DL *ALBUMİN :3.4 MG/DL *T.BİL:6.3 MG/DL *D.BİL :4.8 MG/DL *HB 10.1 G/DL *WBC 9.7X10.000 /Ul *plt 313.000/Ul *ALT 121 IU *T.BİL 6.3 MG/DL *ALBUMİN :3.4 MG/DL *D.BİL 4.8 MG/DL *HB 10.1 G/DL *WBC 9.7X10000 /Ul *PT 20 SN (INR 1.9) *PLT 313.000 /Ul *AST 184 IU *cmv dna pcr < 100copya/ml *T.PROTEİN 5,0 MG/DL Child history- no fauture Family history-no fauture Phisical examination *skin and scleras are icteric *erruption+ *liver 3/1 sm (rigid,smooth) *spleen 2 sm *expitium was long,rales *grade2 ascides *feces dilute *weight: 8100 gr p (50-75) length79sm p(50-75) APPLİCATİON TESTS *ALT 218IU *AST 188IU *ALP 312IU *GGT 59U/L *T.PROTEİN 4.8MG/DL *ALBUMİN 2.8 MG/DL *T.BİL 9.7 MG/ DL *D.BİL 7.1 MG/ DL *HB9.1 G/DL *WBC 6.2X10000/Ul *plt 195.000/ul *PT 22 SN(INR 2.1) *AMMONİA 45 MG/DL Gulnara Heydarova Haydarpaşa Numune Egitim-Araştırma Hastanesi Çocuk Saglıgı Ve Hastalıklıgı Klinigi Case: A Seven month old girl patient Presentinhg complaint: jaundice, acidic feces, skin erruptions. Historiy: jaundice noticed by the familiyin the second month 6 month control ABDOMEN USG:LIVER 109 mm,parankyme heterogenous, spleen 86 mm,gallbladdereyes is present Advanced test *alfa-1-antitrypsine 199 mg/dl *alfafetoprotein 5600ng/ml *eye eximination .pathology(-) *TORCH-S IG M(-) *CMV DNA PCR<100 copy/mı *sweat test :32 meg/l *serum bile acid :293mmol/l *Urinary reducin agent (-) *Urinary succininyl aciton(-) *tandem-MS pataloji(-) *urinary organic acids(-) *blood aminoacids tyrosine and methionin are minimaly high *liver biops can not be made (inr is high) AT 10 TH DAY ,EXİMİNATİON –suction is weak,lethargic *PT 15 SN(INR:1.5) *TORCH IG M(CMV IgM+) - 127 - CONTROL TESTS *ALT 331 IU *AST 217 IU *ALP 288 U/L *GGT 48U/L *T.PROTEİN 4.7 MG/DL *ALBUMİN 2.2 MG/DL *T.BİL 12.5MG/DL *D.BİL 9.3MG/DL *HB 8.8G/DL *WBC 7.3X10000/ Ul *plt 217/Ul *PT32SN(INR2.9) *AMMONİA 184/ ug/DL AFTER K VİT AND TDP THERE İS NO İMPROVOMENT İN PT LEVELS FACTOR V LEVEL:%28 İn the 8 th month the patient was sent tol iver transplantation by the was of patient transport surgery. Allogenic left lateral lobe live transplantation was performed After 2 month of transplantation the patient is being abserved withaut complaint. İn ABCB 11 GEN EXONS C 3084A>G(A1028A) (rs497692) mutation was observedhomologus. The fact that mutation leads to PFIC2 discase is recorded in HUMAN GENE MUTATİON DETABASE. - 128 - INDEX - 129 - A Abdülhamit Tüten 65, 70 Abdulkadir Bozaykut 67, 68 Abdullah Barıs Akcan 40 Abdullah Erdem 100 Abdullah Erdil 44 Abdullah Kocabaş 18 Abdullah Sert 124 Abdullah Yahya Heybeci 94 Abdurrahman Kara 125 Ada Bulut Sinoplu 19 Ádám Schmidt 19 Adem Binnetoğlu 76 Adem Karbuz 19, 29, 30, 51, 61, 66, 125 Adil Umut Zübarioğlu 20, 67, 118, 122 Ágnes Rita Martonosi 20 Ahmet Hakan Gedik 83 Ahmet Naci Emecen 40 Ahmet Sarıcı 99 Ahmet Şenel 37, 53, 54 Ahmet Soysal 121 Ahmet Uçar 83, 122 Aida Koka 50, 59, 60 A.ishak Işık 18 Akın İşcan 26, 28, 82 Akın Savaş Toklu 97 Akl Fahed 23 Alessandro Nicola Abbaticchio 101 Alexander Baranov 65 Alexander Pushkov 65 Ali Adil Fouad 30 Ali Ağaçfidan 122 Ali Alkan 42 Ali Ayata 91 Ali Bay 125 Ali Bülbül 20, 67, 118, 122 Alican Barış 54 Ali Çay 70 Ali Cemal Yumuşakhuylu 76 Ali Gedikbasi 94 Ali Oruç 92 Ali Yurtseven 58 Alkim Beyhan 21 Alper Aykanat 70 Alper Guzeltas 124 Alper Kaçar 19 Alper Soylu 106 Amra Adrovic 19 Anait Gevorkyan 65 Anamaria Ciubara 118 Ancuta Ignat 21, 118, 119, 125 Andrea Párniczky 22 Andrea Szentesi 22 Angelica Cristina Marin 21, 118, 119, 125 Anıl Tapısız 29, 66 Annalisa Resta 101 Anna Tóth 22 A. N. Surkov 18 A. O. Anushenko 18 Aras Tulunoglu 23, 94 Arda Ozyuksel 101 Ariadne Malamitsi Puchner 45 Arif Kut 25 Arzu Aras 24, 70 Asena Pınar Sefer 24, 43, 90 Asli Kibris 123 Aslı Memişoğlu 111, 121, 122 Aslı Nur Ören 92 Aslı Toros 88 Asuman Kıral 24 Atakan Telatar 53 Attila Alp Gözübüyük 71 Avidan Kızılelma Yiğit 41, 126 A. V. Nikitin 18 Avniye Kübra Baskın 25 Ayça Gürbüzer 112 Aycan Unalp 57 Ayça Sözen 25 Aydilek Çakır 82 Aydilek Dağdeviren Çakır 100 Ayhan Taştekin 71, 112 Ayla Balcı 25 Aylin Gençler 75 Aylin Öztarhan 23 Aynur Bedel 24, 26, 70 Ayper Somer 33, 34, 79, 88, 122 Ayşe Ağbaş 55 Ayse Ayzit Atabek 50 Ayşe Bozkurt Turhan 119 Ayşe Çiğdem Aktuğlu Zeybek 37, 47, 65, 68, 107 Ayşe Ferda Ocakci 71 Ayşegül Demir 66 Ayşegül Doğan Demir 26, 54, 88, 92, 105, 106, 119 Ayse Guler Eroglu 59 Ayşe Kalkancı 30 Ayşe Kavak 49 Ayşe Kılıç 63, 109 Aysel Kıyak 49, 113 Aysel Vehapoğlu 26, 27, 28 Ayşenur Kaya 83 Ayse Serap Akgun 100, 101 Ayse Ucar 47 Ayşin Nalbantoğlu 86, 87 Aysun Yahşi 114, 115, 116, 117 B Bahar Çalışkan 61 Bahar Caran 36 Bahar Özcabı 100 Bahattin Tunç 125 Balázs Csaba Németh 22 Banu Bal Çermik 82 Banu Çermik 27 Banu Yazici 28, 94 Başak Koç 33 Başak Saraçoğlu 112 Beáta Rosdy 31, 76 Bedia Deniz Ekici 65 Begum Koc 47 Begüm Şirin Koç 64 Behçet Şimşek 78, 95 Bekir Yiğit Develi 41 Bendavid Matthieu 28 Bengu Ulku Tarhan 107 Benhur Sirvan Cetin 89, 90 Ben Pode Shakked 68 Berna Gümüşoğlu Akşahin 41 Betül Aluç 48 Betül Çınar 29 Beyhan Tüysüz 55 Bidet Philippe 28 Bilge Aldemir Kocabaş 29, 30, 126 Birgül Kırel 39 Birol Ozturk 47 Birol Topçu 84 Bogdan Ciubara 21 Boglárka Fehér 22 Bonacorsi Stéphane 28 Borbala Zsigmond 31 Buket Kara 107 Bülent Çekiç 126 Bulent Hacıhamdioğlu 36, 60, 61, 123, 124 Bulent Oguz Erol 79 Bülent Zülfikar 105 Burak Ardıçlı 81 Burak Deliloğlu 31 Burak Tatlı 34 Burçin Nalbantoğlu 83, 84, 85 Burcu Karadaş 31, 93, 96, 109 Burcu Karakayalı 20, 31 Burcu Karakol 31 Busra Duzyol 28 C Çağatay Günay 95 Çağatay Nuhoğlu 24, 26, 31, 70, 74, 78, 93, 95, 109 - 130 - Cahit Barış Erdur 89 Cana Aksoy Poyraz 69 Canan Aygün 44 Can Demir Karacan 80, 81 Can Polat 38, 39 Caseris Marion 28 Cavid Serdarzade 71 Celal Akdeniz 104 Cengiz Candan 48 Cenk Ermutlu 53, 54 Ceren Çıralı 96 Cicek Bayındır 88 Çiğdem Aktuğlu Zeybek 37, 82, 97, 107 Çiğdem Aydoğmuş 25, 64 Çiğdem Oruç 96 Çiğdem Sağ 74, 95 Çiğdem Ulukaya Durakbaşa 102 Cihangir Akgun 100, 101 Çisem Saygılı 114 Civan Işlak 29 Cointe Aurélie 28 Coşkun Armağan 32 Csilla Andorka 22 D David Terence Thomas 25 Dénes Molnár 22 Deniz Arık 119 Deniz Aygun 32, 33, 34, 35, 50 Deniz Tekin 114 Derya Aysev 30, 115 Derya Büyükkayhan 41, 42, 43 Derya Gültekin Başboğa 35, 36 Didar Arslan 36 Didem Gulcu 69 Didem Ocal Topcu 36, 61, 123 Dilara Ece Toprak 37, 47 Dilara Fucucuoglu 37, 38, 39, 58 Dildar Bahar Genç 40 Dilek Battal 109 Dilek Karacanoğlu 119 Dilşad Koca 26, 70, 74, 93, 96 Dimitris Papamichail 45 Dogukan Aktas 100 Dolunay Gürses 75 Donatella Capodiferro 101 Döndü Nilay Penezoğlu 114 Dóra Mosztbacher 22 Doruk Gül 24, 43, 90 Doygoameneh Ghare Mashggharavi 77 Duhan Demircioğlu 62 Duhan Hopurcuoglu 32 Durdugül Ayyıldız Emecen 40 Duygu Besnili Acar 20, 67, 118, 122 Duygu Övünç Hacıhamdioğlu 25 Duygu Taşkaldıran 85, 86, 87 Duygu Ülger 62 E Ebru Atas Aslan 40 Ebru Eren 121 Ebru Tuğrul Sarıbeyoğlu 111 Ebru Türkoğlu Ünal 20 Ece Gültekin 91, 97 Elif Arslan 41, 97 Elif Begüm Kılıç 82 Elif İnce 114, 117 Elif Kirit 113 Elif Neslihan Tuzcu 104 Elifnur Ildes 107 Elif Yıldız 50 Elif Yılmaz Güleç 23 E. L. Semikina 18 Elvan Çağlar Çıtak 79 Emel Akıncı Ataoğlu 41, 42, 43 Emel Ataoğlu 104 Emel Karaoğlan 48, 76 Emel Torun 82 Emine Ayça Cimbek 73, 74 Emine Erdogan Kutanis 37 Emine Olcay Yasa 43, 48, 102, 103 Emine Polat 80 Emine Tekin 117 Emine Türkkan 41 Emine Yurdakul Ertürk 44 Emine Zeliha Suskan 114 Emin Ünüvar 63 Emmanouela Sdona 45 Emrah Aydın 45, 46 Emre Gök 33 Emre Kelesoglu 100 Emre Ozer 47 Emre Tasdemir 47 Emrullah Arslan 47 Enes Kanay 54 Enes Sali 89 Engin Çarkçı 52 Enise Avcı 48 Ensar Duras 77 Erdal İnce 29, 30, 66, 114, 115, 116 Erdal Sari 108 Erdem Ak 79 Erdogan Soyucen 65 Eren Özek 111, 121, 122 Ergin Çiftçi 29, 30, 66, 114, 115, 116, 117 Erhan Özbek 89 Erkan Çakır 66, 83 Erkan Erfidan 48, 49, 50 Erkut Ozturk 124 Ersin Böcü 50 Ersin Ulu 29 Ertuğrul Kıykım 37, 47, 65, 82, 97, 107 Esin Aldemir 64, 113 Esra Karabiyik 50 Esra Ozcan 38 Esra Özpınar 51 Esra Pehlivanoglu 69 Esra Sesli 51 Esra Yücel 51, 52, 110 Ethem Ayhan Ünkar 52, 53, 54 Éva Kovács 31, 76 Éva Németh 20 Evrim Çeliker Yapıcı 41 Evrim Kıray Baş 20, 67, 118, 122 Eylem Ulaş Saz 58 Ezgi Topyildiz 79 Ezgi Yalçın 54 F Fanni Zsoldos 22 Faruk Öktem 54, 105, 106 Fatih Aygün 55, 98 Fatih Çelmeli 18, 30 Fatih Cemal Özdemir 78 Fatih Demircioglu 107 Fatih Günay 114 Fatih Kara 72, 73 Fatih Kirar 71 Fatih Mehmet Keleşoğlu 112 Fatih Varol 32, 55, 62 Fatma Betül Çakır 56, 93, 105 Fatma Burçin Kurtipek 81 Fatma Deniz Aygün 98 Fatma Ekici 77 Fatma Gamze Demirel 111 Fatma Nur Öz 56 Fatma Özgüç 83, 93 Fatma Özgüç Çömlek 56 Fatma Saltık 57 Fatoş Yalçınkaya 116 Fatouma Khalif Abdillahi 57 Fayize Maden 57 Ferah Genel 89 Ferda Gürkan 58 Feyza Aydın 55, 58 Feyza Coskun 58 Feyza Coşkun 59 Feyza Darendeliler 109, 122 Feyza Mediha Yildiz 108 Feyza Mediha Yıldız 35, 36 Feyza Ustabas 27 Filiz Serdaroglu 57, 90 Firdevs Baş 63, 122 Firuze Erbek Alp 55, 59, 60 Fırat Beğde 30 Foteini Zafeiropoulou 60 Fuat Buğrul 74 Fügen Çullu Çokuğraş 69, 121 Funda Çipe 25 Funda Kökali 61, 79, 80 Funda Öztunç 50, 59 G Gábor Rudas 76 Gábor Veres 22 Gabriela Paduraru 21, 118, 119 Gabriella Kiss 76 Gafur Doğdu 112 Gamze Demirel 71, 111 Gamze Ozgurhan 36, 60, 61, 123, 124 Gamze Sarıkaya Uzan 61 Gamze Yildirim 124 Gizem Höyük 48 Gizem Ondalikoglu 101 Gizem Pamuk 19, 62 Gizem Sayar Bilgin 63 Goar Movsisyan 63 Gökçen Kamış 48, 49, 64, 95 Gökhan Büyükkale 51, 94 Gokten Ongel 60 Gonca Erkose Genc 79 Gonca Keskindemirci 25 Gonca Topuzlu Tekant 34, 121 Gönül Aydoğan 49, 95 Gözde Akın 76 Gözde Yeşil 47, 82, 93 Gözde Yücel 19, 64 Gülay Önal 73 Gulnaz Sariyeva 69 Gül Nihal Özdemir 64 Gülperi Yağar Keskin 40 Gülsen Akay 65 Gülşen Akkoç 121 Gülşen Köse 40, 97 Gülser Esen Besli 102 Gülsüm Güzel 66 Gulnara Heydarova 127 Gulsum Karakus 38 Gunce Basarir 65 Güner Karatekin 65 Gürcan Dikme 47, 62, 64 Gurkan Gurbuz 57 Gurkan Tarcin 65 Guzal Yakhyaeva 65 Güzide Baysan Turanlı 97 Güzide Turanlı 91 G. V. Volynets 18 György Várallyay 31, 76 H Hacer Aktürk 79, 122 Hacer Ergin 96 Hakan Çakır 112 Halil Haldun Emiroğlu 72, 73 Halil Özdemir 29, 66, 114, 115, 116, 117 Halil Suat Ayyıldız 110 Halise Zeynep Işcan 67 Halit Çam 55, 62, 98 Haluk Çokuğraş 32, 33, 34, 35, 50, 46, 98 Haluk Güriz 29 Hamdi Ograg 40 Hamide Sevinç Genç 49, 113 Handan Akoglu 108 Handan Ayhan Akoğlu 67, 68 - 131 - Handan Hakyemez Toptan 65 Hande Kızılocak Kumkumoğlu 35, 47, 62, 64 Haşim Gencer 108 Hasret Ayyıdız Civan 68, 69, 92, 121 Hatice Arıöz Habibi 121 Hatice Dülek 109 Hatice Eren 74 Hatice Gamze Poyrazoğlu 56 Hatice Güllüelli 70 Hatice Nilgun Selçuk Duru 79, 80 Haticenur Kirar 71 Hatice Öztürk 24, 26, 70 Hatice Sınav Ütkü 71 Hatice Ütkü Sınav 111 Havva Evrengül 75 Hayrettin Hakan Aykan 88 Hazal Hazal Huzmeli 71 Helen Bornaun 23, 48, 50, 51, 75, 77, 94, 100 Heves Kırmızıbekmez 36 Hikmet Akbulut 72, 73, 74 Hikmet Tekin Nacaroğlu 59 Hilal Balcı 85, 86, 87 Hilal Fevziye Durmaz Şimşek 74 Hilal Şen 103 Hilal Susam Şen 110 Hilary Pearce 105 Hilmi Apak 35 Hülya Bilgen 111 Hülya Özdemir 111, 120, 121 Hulya Ozturk 107 Hülya Selva Bilgen 120, 121 Huray Kok 74 Hüray Kök 109 Huriye Nursel Elçioğlu 40, 120, 122 Hüseyin Aldemir 48, 76 Hüseyin Aslan 39 Hüseyin Bardak 75 Huseyin Canaz 28, 94 Hüseyin Dindar 116 Huseyin Kaya 38 Hüseyin Kılıç 58 I İbrahim Adaletli 121 Ibrahim Alatas 28, 94 Ibrahim Mert Erbaş 75, 76 İclal Erdem Toslak 126 Ihsan Kafadar 71, 108 Ildikó Guthy 22 İlhan Çiftçi 74 Ilker Ertuğrul 88 Ilker Gonen 113 İlker Tolga Özgen 27, 92 İlter Paydur 74 İpek Akman 120 Irem Ceren Arslan 76 Isa Döngel 57 Ismail Göçmen 25 İsmail İşlek 31 Ismail Mithat Akan 112 İsmail Taşkıran 44 İsmail Yıldız 63 Ismet Çok 109 İsmet Düşmez 24, 43, 90 Issam Cheikh Ahmad 106 István Tokodi 22 J Judit Czelecz 22 Judit Móser 76 Judit Szamosújvári 76 K Kaan Boztug 25 Károly Bakó 22 Katalin Kollár 31, 76 Kazim Coban 57 Lale Sever 99 László Szabó 20 Leda Sianidou 60 Levent Saltık 50, 60 Leyla Besel 78, 124 Leyla Namazova Baranova 63, 65 Leyla Telhan 92, 97, 111 Lida Bulbul 60 Lucrezia De Cosmo 101 Muhammet Ali Varkal 63 Muhammet Demirkol 83, 84, 85, 86, 87 Muhammet Nurullah Yakut 41 Müjgan Alikaşifoğlu 96 Munevver Kaynak Turkmen 40 Murat Elevli 41, 42, 43 Murat Şahin 23, 94 Murat Sarı 76 Murat Sutcu 79, 88 Murat Vural 119 Mürşide Uysal 77 Mustafa Akcam 51, 90 Mustafa Atilla Nursoy 66 Mustafa Behçet Şimşek 78 Mustafa Çakan 48, 49, 113 Mustafa Dilek 107 Mustafa Gülgün 88 Mustafa Güngör 53 Mustafa Hacimustafaoglu 89, 90 Mustafa Kemal Demir 120 Mustafa Kemal Özdemir 19, 41 Mustafa Metin Donma 83, 84, 85 Mustafa Oğur 88 Mustafa Taha Özkul 89 Mustafa Torehan Aslan 89, 90 Mustafa Uzan 61 M N Mahir Tıras 78 Mahmut Can Kızıl 39 Mahmut Doğru 35 Maisam Abu El Haija 22 Maltam Baghirova 78 Manolya Acar 79, 88 Margit Lörincz 19 Mariani Patricia 28 Maria Vasilopoulou 60 Marin Burlea 21, 118, 119 Marton Hernadi 31 Mebrure Yazıcı 66, 88 Mehmet Ali Erceylan 43 Mehmet Atilla Türkmen 106 Mehmet Esat Uygur 52 Mehmet Gunduz 100, 101 Mehmet Karacan 104 Mehmet Kemal Arikan 69 Mehmet Şerif Cansever 37 Mehmet Sinan İyisoy 72 Mehmet Vural 29 Mehmet Yusuf Sarı 56 Mehtap Kağnıcı 89 Mehtap Sak 35 Meliha Aksoy 100 Meliha Demiral 39 Melih Er 79 Melike Emiroğlu 72, 73 Melike Irem Petan 79, 80 Melike Ocak 80, 81 Melis Köse 89 Meltem Erol 37, 38, 39, 58, 59, 78, 124 Meltem Yildirim 60 Merih Çetinkaya 51 Mervan Bekdas 107 Merve Erdemir Kula 82 Merve Hazal Yılmaz 82 Merve Korkut 75, 96 Merve Nur Hepokur 82 Merve Şakar 83, 108 Mesut Dursun 20 Mesut Okur 100, 101 Mesut Parlak 18 Mine Aslan 37 Mine Çalıskan 88 Mine Kucuk 33, 34 Mine Özdil 29 Mine Yüksel 83, 93 Mónika Mellár 76 Müferet Ergüven 48, 50, 90, 102, 103 Muhammed Nurullah Yakut 103 Nadia Al Namat 119 Nadir Koçak 74 Nagehan Aslan 90, 91 Nail Uzunlulu 75 Nalan Karabayır 97, 100 Narin Akıcı 74, 95 Natalia Jurkova 65 Natália Lásztity 22 Nato Vashakmadze 65 Nazan Dalgıç 108 Nazli Kavcik 113 Nedim Samancı 85, 86, 87 Neşe Yaralı 125 Neslihan Özhan 91 Neslihan Tekin 119 Nevin Uzuner 32 Nicola Laforgia 101 Nicoletta Resta 101 Nihat Cem Çelebi 92, 97 Nil Arısoy 99 Nilay Kadakal 92 Nilden Tuygun 80, 81 Nilgün Erkek 101 Nilgün Karadağ 65 Nilgün Selçuk 61 Nilüfer Göknar 27, 54, 119 Nil Yazar Alpay 67 Ninel Revenco 103 Nisa Eda Çullas İlarslan 114 Nisa Pekcan 92 Noémi Scheuring 20 Noémi Vass 22 Nuran Salman 33, 34, 79, 88 Nuray Aktay Ayaz 48, 49, 113 Nur Aydınlı 88 Nur Canpolat 55 Nurcan Yusufoğlu 31, 93 Nurettin Onur Kutlu 28, 88 Nurhan Aruçi Kasap 56, 83, 93 Nuriye Ece Mintaş 85, 86, 87 Nurşen Belet 29 Nursu Kara 28, 94 Nurver Toprak 121 Kazim Oztarhan 100 Kazım Küçüktaşçı 85, 86, 87 Kazım Özatrhan 77 Kazım Öztarhan 23, 51, 94 Kemal Baysal 44 Kemal Gökkuş 30 Kenan Barut 19, 99 Kenan Toprak 95 Kerem Ozel 94 Kirill Savostyanov 63, 65 Kıymet Keçelioğlu Binnetoğlu 76 Konstantin Jerdev 65 Koray Hacıoğlu 77 Kyaw Zin Latt 77, 126 L O Olcay Evliyaoğlu 100 Olcay Yasa 43, 48, 50, 102, 103 Olga Giannouli 60 Olga Gundobina 63 Ömer Bek 30 - 132 - Ömer Ceran 110 Omer Faruk Aydın 117 Ömer Faruk Beşer 41, 52 Ömer Faruk Özer 27 Orhan Özbek 73 Orkide Donma 83, 84, 85 Osen Ari 23, 94, 95 Osman Hacıosmanoğlu 70 Osman Kızılkılıç 99 Osman Umur Başaran 114 Osman Uzunlu 121 Osman Zeki Karakuş 106 Övgü Kul Çınar 96 Oya Ercan 96, 100 Ozan Özkaya 41, 71, 77 Özcan Bör 119 Özden Aksu Sayman 96 Özden Türel 28, 56, 88, 106 Özge Aydemir 39 Özge Besci 111 Özgecan Avcı 67 Ozge Demirel 75 Ozge Gucuyeter 95 Ozgul Yigit 37, 38, 39, 58, 59, 78, 124 Özgün Taban 96 Özgür Genç 67 Özgür Kasapçopur 19, 99, 97 Özgür Kızılca 85, 86, 87 Ozgur Pirgon 51 Özlem Başoğlu Oner 95 Ozlem Bostan Gayret 37, 38, 39, 78, 124 Özlem Erdede 35, 36 Özlem Ketenci Altıkardeşler 70 Özlem Kosvalı 42 Özlem Öçal Doğan 97 Özlem Öner 75 Özlem Özdemir 44 Özlem Sert 101 Özlem Su 66 Özlem Temel 35, 36 Özlem Yapıcıer 120 Özmert Muhammed Ali Özdemir 96 Öznur Vermezoğlu 41, 42, 61, 123 P Pagona Lagiou 45 Pamir Işık 125 Péter Hegyi 22 Pierron Charlotte 28 Pınar Bütün 97 Pınar Karadeniz 118 Pınar Özge Avar Aydın 97, 98, 99, 100 Pınar Selimoglu 124 Pınar Turhan 48 Pınar Yamaç Dilaver 97, 100, 101, 111 Püren Özüdoğru 101 R Rabia Gönül Sezer 67, 68 Raffaella Panza 101 Raif Coşkun 59 Raziye Burcu Güven Bilgin 58 Refia Gözdenur Savcı 102, 103 Rejan Kebudi 105 Rengin Şiraneci 75, 95 Reyhan Dedeoğlu 50, 55, 59, 60 Rıza Rızalar 111 Rodica Eremciuc 103 Royala Babayeva 29 Ruhsar Erdoğmuş 103 Rukiye Eker Ömeroğlu 112 Rukiye İrem Yekeler 104 Rukiye Taş 104 Rumeysa Citli 108 Rumeysa Tuna 105 Rumeysa Yasemin Çiçek 55 Rüveyde Bundak 122 S T Sahin Hamilcıkan 39, 69 Sairah Akbar 105 Şakire Başer 81 Salih Kavukçu 106 Salih Somuncu 120 Salim Çalışkan 99 Saniye Berna Hamilçıkan 41, 52 Sare Betül Kaygusuz 106 Şebnem Özdoğan 83 Sebuh Kuruoglu 47 Seçil Arslansoyu Çamlar 106 Seda Balkaya 48 Seda Şirin Köse 32 Seda Sönmez 106 Sedat Öktem 110, 111, 112 Sedat Özdemir 72 Seda Yılmaz Semerci 51 Sefika Elmas Bozdemir 90 Seher Acar 107 Selçuk Uzuner 88, 106 Selçuk Yüksel 75 Selen Baran 107 Selim Dereci 51, 90 Selim Gökçe 82 Selman Kesici 81 Selver Ametoglou 30 Selvinaz Edizer 57 Sema Bozkaya Yilmaz 108 Sema Saltık 58 Şenay Akbay 91 Şenol Emre 121 Senol Turan 69 Seral Navdar 72, 73 Serdar Ceylaner 18 Serdar Türkmen 26, 27 Serif Cansever 65 Şerife Suna Oğuz 126 Serkan Ozturk 107 Sermin Özcan 40, 83, 108 Serpil Değirmenci 35, 36 Sertac Hanedan Onan 124 Sevgi Yavuz 49, 113 Sevil Arı Yuca 74 Sevil Bilir Goksugur 107 Sevinç Emre 112 Sevinç Gümüş 109 Sevinç Mertoğlu 55 Şeyma Sönmez Şahin 103 Sezen Atik 50 Sezen Ugan Atik 59, 60 Sezgin Şahin 19 Shiyar Hasan 50 Sibel Aşkın Perkin 97 Şifa Şahin 71, 110, 111 Simge Kaya 79 Sinan Oğuz 80, 81 Sinan Uslu 20, 67, 118, 122 Sinan Yavuz 58 Şirin Güven 31 Solmaz Celebi 89, 90 Soner Sazak 41 Songül Yılmaz 115, 116 Suat Ayyıldız 110, 111 Suat Fitöz 66, 115, 116 Süheyla Ocak 110 Süheyla Ocakçı 103 Şükran Poyrazoğlu 109, 122 Sukran Yildirim 94 Şükrü Candan 23 Şükrü Küçüködük 44 Sule Nursah Ayata 31, 109 Şule Terzioğlu 26, 27 Sultan Bent 124 Sultan Kavuncuoğlu 64 Suna Asilsoy 32 Suray Aliskan 123 Suzan Sahin 40 Takis Panagiotopoulos 45 Taliha Öner 118 Tamay Gürbüz 26, 74, 78, 95 Taner Yavuz 31 Tansu Salman 79 Tanyel Zübarioğlu 37, 47, 65, 68, 82, 107 T. A. Skvortsova 18 Tatiana Macovei 103 Taylan Celik 89 Tea Margieva 65 Tekin Bağlam 76 Tevfik Karagöz 88 Tolga Besci 111 Tolga Tüzüner 53, 54 Tuba Koçkar 110, 111, 112 Tufan Kutlu 68, 92, 121 Tuğba Barsan Kaya 39, 119 Tuğba Erat 114, 115, 116, 117 Tuğba Erener 23 Tugba Koca 51, 57, 90 Tugce Goksu Yilmaz 112 Tuğçe Kalaycı Oral 77, 113 Tuğçe Kalın 114 Tuğçe Tural Kara 66, 114, 115, 116, 117 Tuğrul Atay 104 Tülay Erkan 69, 121 Tülin Tiraje Celkan 35, 47, 62, 64, 99 Türkan Uygur Şahin 26, 28, 82, 92 Turkay Rzayev 32, 35 U Ufuk Erenberk 57, 88, 92 Ulkem Colak 117 Ümmühan Öncül 118 Umut Durak 70, 74 V Vahit Guzelburc 100 Vasfi Onur Vermezoğlu 41, 42 Vasile Valeriu Lupu 21, 118, 119, 125 Vefik Arıca 19, 97 Veronika Ila 22 Volkan Tuzcu 104 Y Yağmur Birsev 96 Yair Anikster 68 Yaşar Şen 73, 74 Yasemin Ataman 119 Yasemin Ersözlü 119 Yasemin Kendir Demirkol 120, 122 Yasemin Özdemir Şahan 58 Yasin Şahin 121 Yavuz Gündoğdu 76 Yavuz Köksal 73 Yelda Türkmenoğlu 41, 77, 78 Yelda Türkmeoğlu 97 Yeşim Acar 41, 52, 78 Yeşim Coşkun 120 Yıldız Camcıoglu 34 Yıldız Camcıoğlu 98 Yıldız Perk 29 Yılmaz Yozgat 88 Yusuf Can Doğan 34, 121 Z Zehra Esra Önal 24, 31, 74, 78, 93, 96 Zehra Yavas 33 Zehra Yavaş Abalı 63, 122 Zerrin Onal 60 Zeynep Alp Ünkar 52, 53, 111, 120, 121, 122 Zeynep Birsin Özçakar 115 Zeynep Ceren Karahan 30 Zeynep Emine Tuzcular Vural 109 Zeynep Ersöz Güngör 85, 86, 87 - 133 - Zeynep Gör 52, 126 Zeynep Hızlı 122 Zeynep Işcan 67, 122 Zeynep Karakaya 123, 124 Zeynep Ocak 23, 77 Zeynep Topkarcı 49 Zeynep Yıldız Yıldırmak 40 Ziya Demirci 53, 54 Zoi Beka 60 Zubeyde Kilinc Yaprak 58, 124 Zümrüt Uysal 114 NOTES - 134 - NOTES - 135 - NOTES - 136 - Outlook to Istanbul ORGANIZATION SECRETARIAT TOPKON CONGRESS SERVICES Zühtüpaşa Mah. 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