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Introduction: Mastocytosis is characterized by the excessive proliferation and accumulation of mast cells in organs such as the spleen, liver, lymph nodes, skin, gastrointestinal tract, and bone marrow. Cutaneous mastocytosis, which is only presented with skin involvement, is frequently seen in childhood. Objective: It was aimed to determine the demographic and clinical characteristics of patients with cutaneous mastocytosis and the knowledge level of families about the disease, and to raise awareness about this disease. Material and Methods: The records of 25 cases with cutaneous mastocytosis were evaluated retrospectively and the knowledge levels of the families were measured with a questionnaire. Results: The mean follow-up period of 25 patients was 39 months. The most common type of the disease was urticaria pigmentosa (60%). Itching (n=14) was the most common symptom in patients. Only 2 patients were carrying adrenaline auto-injectors. While 60% of the families (n=15) had fears about the disease; of these 15 families, 53.3% (n=8) were afraid of the re-spread of the disease, 13.3% (n=2) turning of the disease into skin cancer, 13.3% (n=2) occupation of the internal organs, 13.3% (n=2) having a new attack, and 0.4% (n=1) re-forming of the spots. Conclusion: The cutaneous forms of mastocytosis are mostly observed in childhood, and systemic involvement is rare. Mastocytosis progresses with rashes in childhood and its findings are limited to the skin. Families should be better informed about mastocytosis and the need for patients to carry an adrenaline auto-injector due to the risk of attacks should be explained to them., Giriş: Mastositoz, mast hücrelerinin dalak, karaciğer, lenf nodu, deri, gastrointestinal yol, kemik iliği gibi organlarda aşırı çoğalması ve birikmesi ile karakterize bir hastalıktır. Sıklıkla çocukluk çağında sadece deri tutulumu ile seyreden kutanöz mastositoz tipi görülür. Amaç: Kutanöz mastositozlu hastaların demografik ve klinik özelliklerini belirlemek, ailelerin hastalık hakkında bilgi düzeylerini ölçmek ve bu hastalık hakkında farkındalık oluşturmak amaçlanmıştır. Gereç ve Yöntemler: Kutanöz mastositoz tanısı alan 25 olgunun dosyası geriye dönük olarak değerlendirildi ve ailelere kutanöz mastositoz ile ilgili bilgi düzeylerini ölçmek için anket uygulandı. Bulgular: İzlem süresi ortalama 39 ay olan 25 kutanöz mastositozlu hasta çalışmaya dahil edildi. Hastaların %60’ı ürtikerya pigmenzota tipinde idi. Kaşıntı en sık olarak görülen semptom (n=14) idi. Sadece 2 hastamız adrenalin oto enjektörü taşımaktaydı. Ailelerin %60’ında (n=15) hastalık ile ilgili korkular mevcuttu; bu 15 ailenin %53,3’ü (n=8) hastalığın tekrar yayılmasından, %13,3’ü (n=2) cilt kanserine dönüşmesinden, %13,3’ü (n=2) iç organların tutulmasından, %13,3’ü (n=2) atak geçirmesinden, %0,4’ü (n=1) lekelerin tekrar oluşmasından korkmakta idi Sonuç: Mastositoz çocukluk çağında döküntülerle beraber seyreden ve kendini cilt bulguları ile sınırlayan bir hastalıktır. Çocukluk çağında daha çok kutanöz formu görülen hastalığın sistemik tutulumu nadir görülür. Ailelerin mastositoz hastalığı hakkında bilgilendirilmesi ve atak geçirme riskine yönelik adrenalin oto enjektörü taşıması gerekliliği anlatılmalıdır.

Introduction: There are very few studies on the effects of regular blood transfusions on the hemodynamic organization of patients with Beta-Thalassemia Major (BTM). Ambulatory Blood Pressure Monitoring is method that evaluates fluctuations in day-night periods and life cycle changes. In this study, we aimed to investigate the effects of blood transfusion on hemodynamic parameters by the Ambulatory Blood Pressure Monitoring method on the day of transfusion in patients with a diagnosis of Beta-Thalassemia Major. Material and Methods: This study was conducted in patients who were followed up with a diagnosis of BTM between June 2020 and July 2020. The study consisted of 30 patients. The blood pressure of the patients was measured by auscultation method on the morning of the day when they received routine red blood cell transfusion treatments, and the patients were fitted with an Ambulatory Blood Pressure Monitoring device. With Ambulatory Blood Pressure Monitoring, mean systolic blood pressure, diastolic blood pressure, heart rate, mean arterial pressure, values were calculated for each patient. Statistical analysis was performed by the IBM SPSS Statistics 21 package program. The significance limit for the p-value was accepted as Results: There was a significant difference in mean systolic blood pressure and heart rate values between pre-transfusion, transfusion, and post-transfusion periods. In our study, the rate of white coat hypertension was 8.3%, and the rate of masked hypertension was 4.1%. It was observed that 67% of the patients were non-dippers, and the blood pressure burden of one patient was more than 25%. Conclusions: Measurement of hemodynamic parameters with Ambulatory Blood Pressure Monitoring is the gold standard in terms of detection and follow-up of non-dipper patients, indicating increased cardiovascular risk. In practice, Ambulatory Blood Pressure Monitoring should be used more in the follow-up of chronic patients., {"references":["Sadeghi-Bojd S, Hashemi M, Karimi M. Renal tubular function in patients with beta-thalassaemia major in Zahedan, southeast Iran. Singapore Med J. 2008;49(5):410-2.","El-Beshlawy A, El-Ghamrawy M. Recent trends in treatment of thalassemia. Blood Cells Mol Dis. 2019;76:53-58. doi: 10.1016/j.bcmd.2019.01.006.","Sankaran VG, Nathan DG, Orkin SH. The thalassemias. In: Orkin SH, Nathan DG, Ginsburg D, Look AT, Fisher DE, Lux IV S, editors. Nathan And Oski's Hematology and Oncology of Infancy and Childhood. 8th ed. Philadelphia: Saunders; 2014:715-69.","Keser İ. [Molecular studies in hemoglobinopathies]. Turkiye Klin J Pediatr Sci. 2007;3(10):5-10.","Ağaoğlu L, Karakaş Z. Anemias. In: Neyzi O, Ertuğrul T, editors. Pediatrcs. 4th ed. Nobel Medical Bookstores; 2010.","Miri-Moghaddam E, Zadeh-Vakili A, Rouhani Z, Naderi M, Eshghi P, Khazaei Feizabad A. 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Aim: The incidence of pediatric epilepsy ranges between 1-3%, and various anticonvulsant drugs are used in its treatment. Seizure type, effectiveness and side effects are important in drug selection. Various hematological side effects of anticonvulsants have been reported. This study aims to determine the hematological side effects of the anticonvulsants used in our clinic.Methods: A total of 87 epilepsy patients between the ages of 1 and 15 years, who were diagnosed and followed up in the Pediatric Neurology Outpatient Clinic of Health Sciences University, Okmeydanı Training and Research Hospital between January 2017-2018 and who received anticonvulsant medication for at least 3 months were included in this study. The blood values and hematological side effects of the anticonvulsants were noted from the patient files. The statistical analyses were performed with IBM SPSS Statistics v.22 (SPSS IBM, Turkey). Results: Among all, 44.8% of the patients were using Valproic acid (VPA), 28.7%, Levetiracetam (LEV), 12.6%, Oxcarbazepine (OXC), 8%, Phenobarbital (PB), and 4.6%, Carbamazepine (CBZ). Platelet values were below 150,000/ul in 7.6% of VPA users and 14.2% of PB users. The hemoglobin value fell below 10 g/dl in 2.5% of VPA users, 14.2% of PB users, and 8% of LEV users. Absolute neutrophil count fell below 1500/ul in 9% of the patients using OXC and 14.2% of those using PB. The decrease in platelet values before and after anticonvulsant use was statistically significant (P=0.039), while the decrease in hemoglobin and neutrophil values were not. Conclusion: In patients using antiepileptic drugs, complete blood count may be affected. Periodic monitoring of blood parameters is important in the close follow-up of patients. It is not known exactly how and how often the hematological side effects of antiepileptic drugs take place. Future studies on this subject are necessary., Amaç: Çocukluk çağında epilepsi görülme oranı %1-3 arasındadır. Epilepsi tedavisinde çeşitli antikonvülzan ilaçlar kullanılmaktadır. İlaç seçiminde nöbet tipi, ilacın etkinliği ve ilacın yan etkileri önemlidir. Antikonvülzan kullanımının çeşitli hematolojik yan etkileri bildirilmiştir. Bu çalışmanın amacı kliniğimizde kullanılan antikonvülzanlara bağlı hematolojik yan etkileri ve bu etkilerin sıklığını saptamaktır.Yöntemler: Ocak 2017 - Ocak 2018 tarihleri arasında Sağlık Bilimleri Üniversitesi Okmeydanı Eğitim ve Araştırma Hastanesi Çocuk Nöroloji polikliniğinde epilepsi tanısı almış ve en az 3 aydır antikonvülzan tedavi verilmiş, 1 - 15 yaş arasındaki 87 hastanın dosyalarından kan değerleri ve ilaçların hematolojik yan etkileri kaydedildi. Çalışmada elde edilen bulgular değerlendirilirken, istatistiksel analizler için IBM SPSS Statistics 22 (IBM SPSS, Türkiye) programı kullanıldı.Bulgular: Çalışmamızda hastaların %44,8'i Valproik asit (VPA), %28,7'si Levatiresetam (LEV), %12,6'sı Okskarbazepin (OXC), %8'i Fenobarbital (PB), %4,6'sı Karbamazepin (CBZ) kullanıyordu. VPA kullananların %7,6'sında, PB kullananların %14,2'sinde trombosit değerinin 150.000/ul altına düştüğü saptandı. VPA kullananların %2,5'inde, PB kullananların %14.2 'sinde, LEV kullananların %8'inde hemoglobin değerinin 10 g/dl altına düştüğü saptandı. OXC kullanan hastaların %9'unda, PB kullananların %14,2'sinde mutlak nötrofil sayısının 1500/ul altına düştüğü saptandı. İlaç öncesi ve sonrası değerler karşılaştırıldığında, hemoglobin ve nötrofil değerlerindeki düşüş anlamlı bulunmazken, trombosit değerlerindeki düşüş istatistiksel olarak anlamlı bulundu (P=0,039).Sonuç: Anti epileptik ilaç kullanan hastaların kan sayımı etkilenebilmektedir. Hastaların takibinde kan parametrelerinin periyodik olarak yakından izlenmesi önemlidir. Antiepileptik ilaçların hematolojik yan etkisinin nasıl ve ne sıklıkta olduğu tam olarak bilinmemektedir. Bu konuda yapılacak çalışmalara ihtiyaç vardır.

Very recently, a new coronavirus causing a serious, atypical pneumonia cluster that was life-threatening in humans was discovered in December 2019. The virus detected in China spread to 188 countries/regions all over the world in a short period of six months, and it was declared by the World Health Organization to cause pandemics. In Coronavirus Disease-2019 (COVID-19) , the main route of transmission is directly from person-to-person transmission by droplets and contact. In the reported publications, the disease related to Severe Acute Respiratory Syndrome-coronavirus-2 is more common in adults and causes a serious life-threatening disease, especially in the elderly. It is observed that it causes milder clinical presentation in children than adults. Fever and cough are also the most common symptoms in children. In the pandemic course, the department of pediatrics started the training, planning and preparation process before the acceptance of positive patients under the leadership of the Ministry of Health’s constantly updated guide and the established multidisciplinary science committee of our hospital. The treatment of COVID-19 positive pediatric patients has been carried out for most up-to-date and successful manner. The protection both of our healthcare personnel and other pediatric patients who had to use same health care facility during the pandemic course was provided within the framework of the taken infection control measures. While applying the current scientific data and the guide of the Ministry of Health, it was aimed to emphasize that it is important to make decisions in accordance with the dynamics of the process, organized with team spirit.

The longer diagnostic intervals in low- and middle-income countries have been proposed among the possible causes of poorer outcomes in children with cancer. In this single-center study from Turkey, the diagnostic intervals and survival status of 138 children with solid tumors and lymphoma (excluding leukemia) were prospectively evaluated. The median total interval (from the beginning of the first cancer-related symptom to the first day of the cancer-specific therapy), the median patient interval (the time interval from the notification of the first cancer-related symptom to the first admission to a healthcare facility), and the median physician interval (the time interval between the first healthcare admission to the first pediatric oncology visit) were 65, 26, and 24 days, respectively. The estimated 5-year overall survival and event-free survival rates were 80.7% and 69.1%, respectively. The longer time intervals were correlated with age, paternal education, localization, and tumor type. Interestingly, none of the time parameters were found to be associated with survival on regression analysis. In conclusion, the diagnostic delay in children with cancer is multifactorial, and the patient- and disease-related factors are as important as the time intervals on survival.Supplemental data for this article is available online at https://doi.org/10.1080/08880018.2021.1951903.

Background: Henoch-Schonlein purpura (HSP) is a leukocytoclastic vasculitis with small vessel involvement and mainly affects the skin as well as joints, the gastrointestinal system (GIS), kidneys, and, more rarely, other organs. Objective: The objective of this study was to evaluate the sociodemographic characteristics, and clinical and laboratory findings of patients diagnosed with HSP. Materials and Methods: This was a retrospective study done to find out the sociodemographic data, clinical, laboratory findings, and treatment information of patients diagnosed with HSP and was admitted to the Pediatric Clinic of a tertiary care hospital between January 1, 2008, and August 31, 2013. The data were obtained from the hospital’s data processing system. HSP cases were validated according to EULAR/PRINTO/PRES criteria. Mean standard deviation, median, lowest and highest, frequency, and ratio values were used in the descriptive statistics of the data. Results: The study included 85 patients between the ages of 2 and 16 years, wherein 49 patients (57.6%) were male and 36 (42.4%) were female. The mean age was 9.9±3.3 years and 53 patients (62.4%) were under 10 years of age. The most common precipitating factor was upper respiratory tract infections. Purpura was the only symptom observed in all the patients and joint involvement was the second most common symptom (60%). GIS involvement was observed in 46 patients (58.8%) and intussusception was observed in one patient. Nine patients (10.6%) had renal involvement with mild nephropathy. The most frequently observed laboratory findings were increased C-reactive protein (47%) and leukocytosis (31%). Conclusion: HSP is commonly seen in children and leads to life-threatening complications in a minority of patients. Whole patients with GIS and renal involvement should be examined and monitored to assess the severity of the disease and any complications.

Nonalcoholic fatty liver disease (NAFLD) is the most commonly diagnosed liver disease in the recent years, with a prevalence of 15-20% among normal population. Liver steatosis is also a complication of obesity and affects 22-52% of obese children. In this aspect, it is an important public health problem. Increases in the amount of fatty acids entering the liver, increase in fatty acid synthesis and disorders in its secretion are included in its pathogenesis. The relationship between zinc, which is the second most abundant trace element found in the body after iron and necessary for many enzymes to function properly, and fatty liver disease has been shown in previous studies. The aim of this review is to discuss the relationship between zinc and liver steatosis in the light of current studies and contribute to the literature., Non-alkolik yağlı karaciğer hastalığı (NAFLD), prevalansı normal popülasyonda %15-20’lere ulaşan, son yıllarda en sık görülen karaciğer hastalığıdır. Karaciğer yağlanması, obezitenin bir komplikasyonu olarak da karşımıza çıkmakta ve obez çocukların %22-52’ini etkilemektedir. Bu yönüyle önemli bir halk sağlığı sorunudur. Karaciğere gelen yağ asid miktarında artış, yağ asidi sentezinin artışı ve sekresyonundaki bozukluklar, karaciğer yağlanmasının patogenezindeki mekanizmalardan bazılarıdır. Ayrıca, vücutta demirden sonra en bol bulunan ikinci eser element olan ve pek çok enzimin fonksiyon göstermesi için gerekli olan çinko ile karaciğer yağlanması arasında bir ilişki olduğu yapılan çalışmalarda gösterilmiştir. Bu derlemeyi hazırlamaktaki amacımız, vücuttaki çinko miktarının karaciğer yağlanması ile ilişkisini açıklamak ve güncel araştırma sonuçlarını içeren bir veri hazır¬lamaktır. Bu yönüyle çalışmamızın literatüre katkı sağlayacağı kanaatindeyiz.

WOS: 000510640400001, Objective: Infection is a common complication in children with malignancies. There is no consistent guidance for environmental infection control and isolation precautions for neutropenic patients (NP). There are differences between centers. The aim of this questionnaire study was to determine these differences in Turkey. Material and Methods: A multicenter-descriptive questionnaire was conducted on 36 centers from different geografical locations of Turkey. Bone marrow transplantation units were excluded. Each center was contacted at least three-times. Questionnaire was answered by two different doctors from each center. Results: Thirty-six centers including 20 (55.5%) University Hospitals, 12 (%33.3) Research Hospitals, three (8.3%) State Hospital and one Private University Hospital participated in this survey. 94.3% of the centers had a bed capacity of 50 beds and over. Twenty-one (58.3%) centers had pediatric infection ward that followed febrile NP. All centers had an infection control committee. 25% (9/36) of the centers always followed pediatric neutropenic fever patients in a single room. 66.6% (24/36) of the centers had toilet in all patients' room. The door features of patients' room included mostly (94.1%, 32/34) manually opened door. Ten (27.7%) centers had hepa filter system, five of them had positive-negative pressure room. Thirteen (38.2%, 13/34) centers prefered hickmann catheter for accessing a patient's central line. Training was given for catheteter care in all centers. Sixteen (44.4%) centers had determined policies about keeping toys in patient rooms. Visitor restrictions were performed in all centers. None of the centers allowed plants or flowers in hospital rooms. There was a neutropenic diet specific for pediatric NP provided in twenty-seven centers (75%). Conclusion: The prevention and control of infection contributes to the improvement of the prognosis of patients with hematological malignancies. Physicians must be aware of the infection risks and take precautions for infectious complications through the neutropenic period and standard protocols should be established and implemented for patients with hematological malignancies.

PubMedID: 30300449 Objectives: To evaluate the long-term efficacy and safety of deferasirox therapy in a large observational cohort of children with transfusion-dependent thalassemia (TDT) and sickle cell anemia (SCA) in Turkey. Methods: This was a multicenter, prospective cohort study including TDT and SCA patients aged 2-18 years with iron overload (?100 mL/kg of pRBC or a serum ferritin [SF] level >1000 µg/L) receiving deferasirox. Patients were followed for up to 3 years according to standard practice. Results: A total of 439 patients were evaluated (415 [94.5%] TDT, 143 [32.6%] between 2 and 6 years). Serum ferritin levels consistently and significantly decreased across 3 years of deferasirox therapy from a median of 1775.5 to 1250.5 µg/L (P

Introduction: In this study, we aimed to investigate the frequency and clinical features of respiratory syncytial virus RSV infection among infants hospitalized with the diagnosis of lower respiratory tract infection.Materials and Methods: The study was conducted between 1 December 2012 and 1 March 2013 in our pediatric clinic. A total of 170 children between 1-24 months of age with lower respiratory tract infection were included. Their clinical and laboratory findings were analyzed retrospectively. The detection of RSV antigen was carried out by immunoassay method on nasopharyngeal swabbing samples.Results: RSV infection was determined in 37.1% of the patients. RSV + patients under the age of 6 months was statistically significantly higher than RSV - patients. In RSV + and RSV - patients there was no statistically significantly difference in gender, infiltration in chest radiography, C-reactive protein positivity

OBJECTIVE: The aim of this study is to evaluate the relationship between iron deficiency anemia IDA , a common disease of childhood, and thyroid hormones.METHODS: This study was carried on 49 children with iron deficiency anemia IDA and control group of 27 healthy children who were admitted to Well Baby Clinic and outpatients clinic of Pediatric Hematology and Oncology Department of M.O.H. Okmeydanı Research and Education Hospital between January 2011 and July 2011. Blood samples for total blood count, blood iron, total iron binding capacity TIBC and ferritin levels were obtained from patients. Afterwards, blood were driven from patients with IDA and blood levels of thyroid stimulating hormone TSH , triiodothyronine T3 , thyroxine T4 , free T3 FT3 , free T4 FT4 were measured.RESULTS: Within demographic characteristics, history of premature birth, duration of breast feeding, the onset age of supplementary nutrition, onset age of cow milk feeding, daily milk consumption and weekly meat consumption of two groups were assessed. No statistical significance was found between two groups in terms of positive history of premature birth, duration of breast feeding, the onset age of supplementary nutrition, onset age of cow milk, daily milk consumption p>0,05 . Weekly meat consumption of children in control group was found to be higher compared to anemic group and this constitute a statistical significance p=0,009 . Mean values and standard deviations for hematological parameters were calculated for both groups individually. In both groups, levels of TSH, TT3, TT4, FT3, FT4 were compared in order to assess thyroid functions and find if there were any difference. No statistical significance was found p>0,05 . CONCLUSION: In conclusion; several biochemical and morfological alterations occur in children with IDA. In our study no statistical significance was found between patients in pediatric IDA group and healthy children in control group for thyroid hormone levels., AMAÇ: Bu çalışmada, çocuklarda sık görülen hastalıklardan biri olan demir eksikliği anemisi DEA ve tiroid hormonları arasındaki ilişkinin araştırılması amaçlanmıştır. YÖNTEMLER: Bu çalışma, Ocak 2011 - Temmuz 2011 tarihleri arasında S.B Okmeydanı Eğitim ve Araştırma Hastanesi Sağlam Çocuk Polikliniği ve Çocuk Hematoloji Polikliniği’ne başvuran DEA saptanan 49 hasta çocuk ve kontrol grubunu oluşturan sağlıklı 27 çocuk olmak üzere toplam 76 çocuk üzerinde gerçekleştirildi. Polikliniğe başvuran hastalardan hemogram, demir, Total demir bağlama kapasitesi TDBK , ferritin düzeyi için kan alındı. Sonrasında DEA saptanan ve kontrol grubunu oluşturan sağlam çocuklardan kan alınarak Tiroid stimulan hormon TSH ,triiyodotironin T3 , tiroksin T4 , serbest T3 FT3 , Serbest T4 FT4 hormon düzeyleri çalışıldı.BULGULAR: Çalışmaya alınan iki grup arasında hastalara ait demografik özelliklerden, prematüre doğum öyküsü, anne sütü alım süresi, ek gıda başlanma zamanı, inek sütü başlama yaşı, günlük süt tüketimi, haftalık et tüketimi incelenmiştir. Gruplar arasında, prematüre doğum öyküsü varlığı, anne sütü alım süreleri, ek gıda başlangıç süreleri, inek sütü başlama zamanları ve günlük süt tüketimi arasında istatistiksel olarak anlamlı fark saptanmamıştır p>0,05 . Kontrol grubundaki çocukların haftalık et tüketiminin, anemi grubuna göre daha fazla olduğu gözlenmiş ve iki grup arasındaki fark istatistiksel olarak anlamlı bulunmuştur p=0,009 . Hematolojik parametrelerin ortalama değerleri ve standart sapmaları iki grup için de ayrı ayrı hesaplanmıştır. Her iki grupta da tiroid fonksiyonlarını değerlendirmek ve fark olup olmadığını saptamak amacıyla TSH, TT3, TT4, FT3, FT4 değerleri karşılaştırılmış, iki grup arasında istatistiksel olarak anlamlı bir fark olmadığı saptanmıştır p>0,05 .SONUÇ: Sonuç olarak; demir eksikliği anemisi olan çocuklarda bir çok biyokimyasal ve morfolojik değişiklikler olmaktadır. Bizim çalışmamızda pediatrik yaş grubunda DEA grubu ve kontrol grubundaki sağlıklı çocuklarda, tiroid hormon düzeyleri arasında anlamlı fark saptanmamıştır.

Granisetron is a safe and effective prophylaxis for nausea and vomiting associated with moderate to highly emetogenic chemotherapy. Few trials have been conducted to determine the optimal effective dose of granisetron in children with cancer. The objective of this report was to compare two doses of granisetron in patients with optic pathway tumors receiving moderately emetogenic doses of carboplatin.In this double-blind, crossover, randomized study, antiemetic efficacy and tolerability of two dose levels (10 and 40 microg/kg) of granisetron in the prevention of acute and delayed nausea/emesis were compared in children and young adults. A total of 18 patients (13 boys) aged 1-23 years (median 7.7 years) treated with a moderately emetogenic dose of carboplatin were randomly assigned to receive either 10 or 40 microg/kg of slow granisetron intravenous (i.v.) infusions at alternating cycles of chemotherapy in a blinded fashion until the end of the study period or until their chemotherapy regimen ended. In this way, the patients acted as their own controls.Patients in the granisetron 10 and 40 microg/kg groups received 104 and 121 cycles of chemotherapy, respectively. There was no significant difference in antiemetic efficacy in terms of nausea and emesis between the dose groups in the first 5 days of chemotherapy. The treatment was well tolerated.We conclude that granisetron 10 and 40 microg/kg have comparable efficacy in controlling carboplatin-induced acute and delayed nausea/emesis and is well tolerated in children and young adults.

Purpose: Kawasaki disease is common in children and is an acute systemic vasculitis affecting coronary arteries. Kawasaki disease has multisystemic nature with a variety of presenting symptoms. Without treatment 20- 25% of Kawasaki disease patients develop coronary artery aneurysms. Our goal is to summarize the clinical data of patients treated with Kawasaki disease at our clinics. Material and Methods: Clinical features, laboratory findings, diagnosis and treatment of 21 patients with Kawasaki disease hospitalized at Turkish Ministry of Health Okmeydanı Training and Research Hospital, Clinic of Pediatrics during January 2009 to January 2015 were retrospectively evaluated.Results: The patients were between 9 and 67 months old (median 27.52± 18.78), 71.4% (n:15) were male. The duration of disease before diagnosis was 7.1±3.48 days, median 6 days. All patients had fever. The most common clinical features were oral cavity changes 95.2% (n:20), rash 85.7% (n:18), conjunctival injection 71.4% (n:15), extremity changes 61,9% (n:13), cervical lymphadenopathy 57.1% (n:12). We also observed sterile pyuria in 33.3%, desquamation of perianal area 28.6 %, aseptic meningitis in 9.5%, hydrops of the gallblader 4.8%. 19.1% patients had incomplete Kawasaki disease. 6 patients, 28.5% had coronary artery abnormalities. Intravenous immunglobulin treatment failure was observed in 9.5% of patients, one of them was diagnosed as macrophage activation syndrome.Conclusion: Kawasaki disease has difficulty in diagnosis because of having broad spectrum of presenting symptoms. Early diagnosis and treatment is very important in preventing coronary artery abnormalities., Amaç: Kawasaki hastalığı çocuklarda görülen, farklı semptomlarla ortaya çıkan, koroner arterleri tutan akut multisistemik bir vaskülittir. Tedavi edilmeyen hastaların %20-25’inde koroner arter anomalisi gelişir. Burada amacımız hastanemizde Kawasaki hastalığı nedeniyle tedavi verilen hastaların klinik sonuçlarını değerlendirmektir.Gereç ve Yöntem: SB Istanbul Okmeydanı Eğitim ve Araştırma Hastanesi Çocuk Sağlığı ve Hastalıkları Kliniğine Ocak 2009 ile Ocak 2015 arasında Kawasaki hastalığı nedeniyle yatırılan hastaların klinik ve laboratuvar bulguları, tanı ve tedavileri sonuçları arşiv dosyalarından retrospektif olarak değerlendirildi. Bulgular: Yaşları 9 ile 67 ay arasındaki 21 Kawasaki hastalığı olgusunun yaş ortalaması 27.52±18.78 idi. Hastaların 15 (%71,4)’ü erkekti. Tanı alma süresi 7.10±3.48 ortalama 6 gün idi. Tüm hastalarda ateş bulunmaktaydı, oral kavite lezyonları %95.2 (n:20), döküntü %85.7 (n: 18), konjonktival injeksiyon %71.4 (n:15), ekstremite değişiklikleri % 61.9 (n: 13) ve lenfadenopati %57.1 (n:12) olguda görüldü .Ayrıca olguların %33.3’unda steril pyüri, perianal deskuamasyon %28.6’sında, %9.5 ‘unda aseptik menenjit ve %4.8 hastada safra kesesi hidropsu gözlendi. Hastaların %19.1‘i inkomplet Kawasaki hastalığı tanısı aldı. Koroner arter anomalisi %28.5 (n:6) hastada görüldü. İntravenöz immunglobulin yanıtsızlığı %9.5 (n: 2) hastada izlendi, onlardan birine makrofaj aktivasyon sendromu tanısı konuldu.Sonuç: Kawasaki hastalığı farklı klinik görünümleri olması nedeniyle tanı zorluğu olan bir hastalıktır. Koroner arter anomalilerini önlemek için hastalığın erken tanı ve tedavisi çok önemlidir.

In the Stockholm Declaration, it states that " The emergency in terms of medicine is characterized by the acute and unforeseeable imbalance between the capacity and sources of the medicine profession within a certain period of time and the requirement of people affected by the emergency situation or people whose health are under threat." Since potential exposure from the inner and outer stimulus is higher for the organism that is developing in physiological and psychological sense, childhood period is a risky period without considering causation. All of the risks cover the child, family and society. In risk evaluation, live, protection, development and participation rights of child shall be basis in humane and legal sense. Considering the fact that child is the subject of the social life, the damages that may happen should be realized before creating domino effect and injuries should be treated and they should be prevented with precautions that are the dynamo of themselves. Convention on the rights of children gives right to all of us in particular to pediatricians like us to warn and remind states of their duties in the event of failure to abide by these rights to protect the children from risky situations. Reasonable and practical approaches shall be produced with evaluations conducted in the scope of the health, security and education in case of emergency. J Clin Exp Invest 2015; 6 (3): 324-330

Nous presentons un cas de resolution d'une sous-occlusion intestinale chez d'un garcon de six ans atteint d'un lymphome non hodgkinien (LNH) refractaire obtenue sans intervention chirurgicale grâce a un traitement par Taxol®. En l'absence de reponse au traitement standard de premiere intention du LNH (cellules B de stade 3) et a la chimiotherapie de deuxieme intention, un protocole de troisieme intention associant une radiotherapie et une chimiotherapie etait initie au cours duquel le patient etait hospitalise pour sous-occlusions par progression tumorale. L'adjonction de Taxol® (200 mg/m 2 /semaine) sans interruption du protocole a permis l'involution totale de l'ileus avec reduction de la masse tumorale dans les 24 h sans intervention chirurgicale. L'administration de Taxol® etait poursuivie pendant six cycles sans effet secondaire ou toxicite importante. Le TDM de controle montrait une reduction significative de la tumeur. Une thrombopenie, survenue apres le cycle suivant, n'a pas pu etre controlee et le patient est decede d'une reprise tumorale avec dissemination. On trouve dans la litterature quelques rapports de traitement de LNH refractaire par Taxol® chez l'adulte, notre cas serait le premier rapporte chez un enfant. En vue de l'efficacite et de la tolerance observees chez notre patient, l'administration de Taxol® serait licite pour le traitement de LNH refractaire chez les enfants afin d'ameliorer la survie sans sacrifier la qualite de vie. Des etudes chez un plus grand nombre de patients seraient necessaires pour conclure concernant l'efficacite reelle du Taxol® chez les enfants atteints de lymphome non hodgkinien refractaire.

WNT signaling has been implicated in the regulation of hematopoietic stem cells and plays an important role during T-cell development in thymus. Here we investigated WNT pathway activation in childhood T-cell acute lymphoblastic leukemia (T-ALL) patients. To evaluate the potential role of WNT signaling in T-cell leukomogenesis, we performed expression analysis of key components of WNT pathway. More than 85% of the childhood T-ALL patients showed upregulated beta-catenin expression at the protein level compared with normal human thymocytes. The impact of this upregulation was reflected in high expression of known target genes (AXIN2, c-MYC, TCF1 and LEF). Especially AXIN2, the universal target gene of WNT pathway, was upregulated at both mRNA and protein levels in similar to 40% of the patients. When beta-CATENIN gene was silenced by small interfering RNA, the cancer cells showed higher rates of apoptosis. These results demonstrate that abnormal WNT signaling activation occurs in a significant fraction of human T-ALL cases independent of known T-ALL risk factors. We conclude that deregulated WNT signaling is a novel oncogenic event in childhood T-ALL.

Introduction: Iliopsoas hematoma is a serious, life threatening bleeding event in patients with hereditary clotting disorders and significantly associated with morbidity. It occurs most commonly in patients with severe hemophilia (Balkan et al Haemophilia 2005). In this review, we evaluated our patients with iliopsoas hematoma and compared with literature. Methods: The eleven pediatric patients with hemophilia and iliopsoas hematoma treated in a research and training hospital in Istanbul, between 2005 and 2015 were enrolled. The clinical characteristics including symptoms, signs, complications, and treatment were analyzed retrospectively. Results: We evaluated 14 episodes of iliopsoas hematoma from 11 patients (10 hemophilia A and 1 hemophilia B). All patients were male and the median age was 12 (8 -14) years old. Eight patients had one episode, three had two episodes. One patient had a high titer inhibitor against factor VIII. The hematoma was spontaneous and unilateral in all cases. The most common symptoms were hip pain and hip flexion contracture. Iliopsoas hematomas were confirmed by ultrasound, CT or MR scan. The treatment consisted of factor replacement and rehabilitation therapy. One patient needed erythrocytes transfusion. The mean duration of factor replacement therapy was 13.5 +/- 2.1 days. One severe hemophilia A patient with inhibitor treated with recombinant factor VIIa. Long term complications included paresthesia in 3 patients in the distribution of femoral nerve, quadriceps atrophy in 3 patients and permanent abnormal posture in one patient. Conclusion: The psoas muscles are located in the iliopsoas compartment posterior to the transversalis fascia, which is the posterior boundary of the retro peritoneum. Presentation of iliopsoas hematomas is often non-specific with abdominal, pelvic, back or groin pain or swelling. If they are large, iliopsoas hematomas can present with constipation, urinary frequency or fever. They can even compress the femoral nerve and cause a femoral neuropathy . A delay in treatment may result in permanent femoral nerve palsy (Mannucci N Engl J Med 2004). In this report, we present our experience and treatment modality in 14 episodes of iliopsoas hematoma in pediatric hemophilia patients. Rehabilitation treatment with factor replacement is safe and effective therapy for patients with small hematomas and little or no neurological symptoms. More aggressive recommendations have been made for patients with large hematomas and severe neurological symptoms or hemodynamic instability. An early diagnosis allows early factor replacement therapy, and decreases the risk of recurrence and morbidity. Disclosures No relevant conflicts of interest to declare.

Introduction: The survival of the patients with thalassemia has progressively improved with advances in therapy, however, increased survival allowed for several complications of disease. Metabolism of vitamin D affected in patients with thalassemia, due to accumulation of iron in the liver as the skin. The aim of this study is to compare vitamin D and parathyroid hormone (PTH) levels between patients with thalassemia and healthy control group. Methods: In pediatric hematology clinic, 33 patients (23 male, 10 female) with beta thalassemia major and 33 age-sex matched healthy people as a control group were included. Serum 25- OH Vitamin D levels were defined as normal >30 ng/ml, insufficiency between 20-30 ng/ml and deficiency < 20 ng/ml and PTH levels as normal between 10-65 pg/ml, decreased 65 pg/ml. Results: Mean age was 23.02+8.18 years old in patient group and 23+ 8.16 in control group. %33.3 of the patients had 25-OH vitamin D insufficiency and %30.3 of had 25-OH vitamin D deficiency. In different studies, the 25-OH vitamin D deficiency found between %12-90, and insufficiency between % 24.7-69.8 of the thalassemic patients. %81.8 of the patients had normal PTH levels, %18.2 decreased and none of the patients were found increased. In literature, there were confluent results about PTH levels in thalassemic patients, some of the studies showed hypoparathyroidism, and some of hyperparathyroidism or normal levels of PTH (Vogiatzi Br J Haematol 2009, Moulas Acta Paediatrica 1997, Merchant Indian J Pediatr 2011). In our study we couldn't show hyperparathyroidism in patient group. Also we found parathyroid hormone levels were significantly lower (p Disclosures No relevant conflicts of interest to declare.

It is generally accepted that seroconversion of hepatitis B virus (HBV) surface antigen (HBsAg) to an antibody to HBsAg (HBsAb) indicates clearance of HBV. The reverse seroconversion to hepatitis B virus infection has been sporadically described in hematological patients receiving cytotoxic therapy and can be associated with the development of acute hepatitis. Here we report a 14-year-old male patient who developed HBV reactivation after intensive chemotherapy containing fludarabine for second bone marrow relaps of acute lymphoblastic leukemia. Fludarabine is a chemotherapatic agent that used for the treatment of relapsed acute leukemia in pediatric patients. He was HBsAg(−)/anti-HBs(+) and with normal ALT levels before intensive chemotherapy (fludarabine, dexamethasone, GCSF, alexan)for treatment of second bone marrow relaps. 6 weeks after completing two cyclus of chemotherapy with fludarabine, the patient developed transaminitis and evidence of active HBV infection (the patient had test results positive for HBsAg, negative for HBsAb, and positive for HBV DNA). the serum levels of aminotransferases were increased progressiveley. Lamivudine was administered immediately after confirming the HBV reactivation, which considerably improved jaundice, aminotransferase levels and serum HBV DNA titers after one month. The patient was able to resume the chemotherapy under continuis lamivudine treatment. This case indicates the need for physicians to be aware of fludarabine-related HBV reactivation, closely monitor and detect reactivation early, even in an HBsAg-negative patient.

Chronic idiopathic (immune) thrombocytopenic purpura (ITP) develops in approximately 20% of children with acute ITP. The therapies for chronic refractory ITP include splenectomy, steroids, intravenous immunoglobulin (Ig), anti-D Ig, monoclonal antibodies, danazol, chemotherapy, plasma exchange, and others. We report five 5 RhD-positive, pediatric,, non-splenectomized patients with chronic immune thrombocytopenic purpura (ITP), treated with anti-D. Patients were refractory to steroids and high dose intravenous immunoglobulin (IVIG). The mean age of the patients was 10.5 years, duration of thrombocytopenia was more than 6 months, and the median platelet count was 6x 10(9)/l. Patients received a single intravenous dose of 75 microg/kg anti-D, and were monitored for response to treatment and adverse events. Complete blood cell and renal function tests were assessed at baseline and 1, 7, 14 and 28 days after infusion. By 24 hours after treatment two of patients had achieved a platelet count>20 x 10(9)/l. Mild headache, fever, or chills occurred in one patients and ameliorated with prednisone and acetaminophen premedication. None of the patients had more than 0.5 gm/dl fall in the hemoglobin level and renal dysfunction following anti-D infusion by day 1,7, 14, 28. A single 75 microg/kg dose of anti-D may increase the platelet count in some children with chronic resistant immune thrombocytopenic purpura with an acceptable safety profile. It is a valuable, safe, well tolerated, and cheap alternative to conventional chronic ITP treatments in selected non-splenectomized patients.

Background: Microbial killing killing of by phagocytic cells by iron containing myeloperoxidase pathway is part of the immune system. We aimed to investigate the effects of iron deficiency anemia (IDA) on oxidative burst response of phagocytic cells and understand if the effect is reversible after iron supplementation. Material and methods: There were 57 IDA patients and 31 healthy children as the control group that were aged between 6 months-12 years with similar demographic status. IDA patients were classified according to the severity of their anemia as follows; group 1 (Hb10 gr/dl). Neutrophil and monocyte oxidative burst response of each three groups of IDA and control group were compared by flow cytometry. IDA group were given oral iron supplementation (3 mg/kg-Ferrosanol) therapy. On day 15 of iron supplementation therapy neutrophil and monocyte oxidative burst response of each three groups of IDA were rechecked and compared to the control group. In order to minimize the discrepancies observed in functional studies, the oxidative burst index was calculated as the proportion of oxidative burst response in IDA group to the simultaneous oxidative burst response in the control group. Results: The number of infection episodes observed in the last three months was significantly higher in the IDA group (0–13 episodes) than the control group (1–2 episodes) (p=0.05). Neutrophil (p=0.1) and monocyte (p=0.02) oxidative burst responses in IDA group were found to be less than the control group. On day 15 of iron supplementation therapy, these values were observed to increase to the control group’s values. Evaluation of neutophil and monocyte burst indexes at the diagnosis of IDA revealed that the neutrophil burst indexes in the Hb ≤10 gr/dl group (p=0.006), and monocyte burst indexes in the Hb Conclusion: Correction of the differences in oxidative burst response after iron supplementation therapy implies the fact that IDA might be the reason for changes in neutophil and monocyte burst indexes.