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A tumor with EWSR1/FLI fusion displaying extensive well differentiated neuroblastomatous differentiation is presented.A nine-year-old female patient had a thoracic vertebra 8 paraspinal mass. The lesion was resected incompletely. Histopathologically, a small round cell tumor with gangliomatous differentiation was seen. This was initially diagnosed as an intermixed ganglioneuroblastoma. In the completion surgery biopsy material, the small round cell component was more prominent. Immunohistochemistry for both samples showed membrane positivity for CD99 and nuclear positivity for NKX2.2 in the small round cell component of the tumor. Molecular analysis revealed EWSR1/FLI fusion. The diagnosis then considered a "Ewing Sarcoma Displaying Extensive Well Differentiated Neuroblastomatous Differentiation".Tumors with the EWSR1/FLI fusion may show neuroblastomatous differentiation. We chose to treat this as an Ewing Sarcoma (ES). Recognition of this phenomenon in ES cases may prevent a possible misinterpretation and a failure in oncologic treatment.

The Beckwith–Wiedemann spectrum (BWSp) ranges from isolated lateralized overgrowth (ILO) to classic phenotypes. In this broad clinical spectrum, an epigenetic alteration on chromosome 11p15.5 can be detected. The risk for embryonal tumors is high, especially in patients with lateralized overgrowth (LO). The aim of this study is to investigate epigenetic alterations in 11p15.5 and tumor risk in 87 children with LO. The methylation level of 11p15.5 was examined in the blood of all patients and in skin samples or buccal swabs from 40 patients with negative blood tests; 63.2% of patients were compatible with the ILO phenotype, 18.4% were atypical, and 18.4% were classic. The molecular diagnosis rate was 81.2% for the atypical and classic phenotypes, and 10.9% for the ILO phenotype. In patients with epigenetic alterations, LO was statistically significantly more severe than in test negatives. Tumors developed in six (6.9%) of the total 87 patients with LO; four belonged to the atypical or classical phenotype (12.5%) and two to ILO (3.5%). Three of the four patients with atypical/classical phenotypes had pUPD11, one had IC1-GOM alteration, and two ILO patients were negative. We conclude that LO patients should be monitored for tumor risk even if their epigenetic tests are negative.

Background: A novel type of Coronavirus emerged at Wuhan in late 2019 involving preferentially the respiratory system. Owing to the rapid spread, almost 22 million people became infected and 700,000 died. Similar to other countries, the need for additional hospital beds and intensive care units required diversion of health care resources toward the care for those with COVID-19 in Turkey. Telemedicine appeared as a safe and low-cost alternative for the maintainability of pediatric health services during the pandemics. Within this context, we aimed to deliver the health services through telemedicine during the follow-up of chronic childhood diseases. Materials and Methods: This prospective study included five pediatric subspecialties, including allergy immunology, hematology and oncology, nephrology, rheumatology, and inborn metabolic disorders. After the interview, patients and involved physicians were requested to fill out a questionnaire designed to measure the level of satisfaction and the quality of the service we offered. Results: Of the 263 interviews, overall patient and physician satisfaction was 99% and 87%, respectively. As results of the interviews, 250 routine visits were performed, 181 acute complaints were assessed, drug changes were made in 118 patients, 9 patients were determined to be unable to get their drugs, and 12 who misused their drugs. The main advantage of the telemedicine declared by the patients was "not to waste time for transportation." The main concerns of the participants were inability to perform physical and laboratory examinations. Conclusion: Consequently, we considered telemedicine as a feasible alternative not only during pandemics but also in daily practice in Turkey.

Objective: Neurofibromatosis (NF) is the most common autosomal dominantly inherited neurocutaneous syndrome. The characteristic features of NF type 1 (NF-1) are café au lait spots, axillary and inguinal freckling, peripheral neurofibromas, optic pathway glioma, and Lisch nodules. The present study aimed to analyze the clinical features of children with NF-1. Materials and Methods: In this study, the children with NF-1 diagnosed and followed-up in our center between 2000 and 2020 were retrospectively evaluated. Demographic and clinical features of patients were defined. Results: The study group consisted of 52 patients. Of those, 25 were boys and 27 were girls. The children’s median age at diagnosis was 5.9 years (1-15.8). Café au lait (CAL) spots and axillary/inguinal freckling were observed in 50 and 24 patients, respectively. Neurofibroma was present in 22 cases. Ten of the cohort had optic gliomas, and 39 of them had cranial hamartomas. Orthopedic complications such as scoliosis, tibial pseudoarthrosis, and osteoporosis were observed in 13 patients. Eleven children had neurocognitive disorders. Conclusions: Early diagnosis is important in neurofibromatosis to prevent the complications of the disease. Also, neurological development and secondary malignancy follow-up should be done carefully in this group of patients.

Objective Difficulties encountered in the diagnosis and treatment of vascular anomalies located in the extremities of the children. The most common vascular lesions are hemangiomas and venous malformations. The complex malformations, such as, Klippel-Trenaunay Syndrome are much less commonly encountered lesions. Treatment of vascular malformations are variable based on the etiology of the lesion and clinical presentation. In this study, we aimed to share our experience on the clinical features of vascular lesions in the extremities of the children. Material and methods The demographic, clinical and prognostic features of 330 children with vascular anomalies followed at IUC, Cerrahpasa Medical Faculty, Department of Pediatric Hematology and Oncology were retrospectively reviewed. Fifty-one patients with lesions >5 cm in diameter were included into the study. The diagnosis, age, sex, history of prematurity, lesion type and location, imaging and biopsy findings, complications, details of treatment, and follow-up were evaluated. Results Twenty-nine (57%) of patients were female and 22 (43%) were male. The female to male ratio was 1.3:1. The median age at admission was 15 months (10 days-180 months). Eight patients (16%) had a history of premature birth. Thirty-one patients (61%) had lesions since birth, eight lesions (8%) appeared in the first month of life and 6 (12%) occurred after 1 year of age. Sixteen of the patients (31%) had hemangioma, 11 (22%) had lymphangioma, 19 (37%) had venous malformation and 5 (10%) were diagnosed as Klippel Trenaunay Syndrome. The lesions were in the upper extremity in 21 patients (41%), in the lower extremity in 27 patients (53%), and both lower and upper extremities were affected in 3 patients (6%). Of all patients, six had intramuscular and two had intraarticular lesions. The diagnosis was made on clinical grounds in most of the cases. In 22 children Magnetic Resonance Imaging was performed for differential diagnosis and to demonstrate the infrastructure of the lesion and the extent of local infiltration. Histopathologic examination by biopsy was done in four patients. Complications developed in 19 patients as follows: Disseminated intravascular coagulation in 6, bleeding in 4, thrombosis in 3, and soft tissue infection in 6. Twenty-one patients were not given any treatment. Medical treatments were propranolol in 14 patients, sirolimus in 4 patients, propranolol and sirolimus in 5 patients. Intralesional bleomycin injection was performed in 3 children. Conclusion The diagnosis, classification and treatment of extremity located vascular malformations in children are complex. Treatment strategy should be defined as in accordance with a combination of the type of the vascular malformation, the age of the patient and the clinical picture.

Objective: After chemotherapy, cancer survivors suffer from acquired immunological defects and become vulnerable to vaccine-preventable diseases. There are no universally approved revaccination guidelines for non-transplanted oncology patients. This study aimed to share our experience of revaccination in childhood cancer survivors to plan future vaccination schedules. Materials and Methods: This retrospective study was conducted in a Pediatric Oncology Department of a university-affiliated hospital. Patients who were diagnosed with malignancy other than leukemia constituted the study population. Patients were directed for revaccination 6 months after the cessation of treatment. Revaccination was performed according to patients' vaccination status before chemotherapy and seronegativity. Results: Of the 64 patients in the study, 44 (68.75%) were boys. The mean age at the time of diagnosis and at start of vaccination was 8.8 +/- 5.3 years and 10.6 +/- 5.1 years, respectively. Hodgkin's lymphoma was the most common diagnosis. The vaccination schedule of 7 patients was interrupted because of chemotherapy; after completing the missing vaccine doses, the serology of 2 patients was negative for at least 2 antigens. The vaccination schedule of 57 patients was completed before beginning chemotherapy and 52 of them were seronegative for at least 1 antigen. No adverse reactions or life-threatening infections were observed because of vaccinations. Conclusion: There are different approaches when vaccinating the oncology patients after chemotherapy. Watching out for the four touchstones mentioned in our study will protect the patient and do no harm. More studies are needed to constitute universal and standardized revaccination guidelines for these patients.

Background: Proper clinical use of blood and blood products requires competent theoretical and practical knowledge of transfusion medicine. The Curriculum Development and Standard Determination System Medical Specialization Board is prepared Hematology Specialist Education Core Curriculum in Turkey. In this study, we aimed to determine the access of hematologists to the learning objectives defined by curriculum for the transfusion medicine and the factors affecting it.Methods: Hematologists who have been members of Turkish Hematology Society since 2013 have been included in the study, The survey questions were prepared based on the curriculum for transfusion medicine. The study was applied to hematologists with "survey monkey" application. The questionnaire consisted of a competence self-assessment with Likert scale and theoretical multiple-choice knowledge questions. Results: Of the 213 hematologists, 54 (25%) were included in the study. Hematologists rated their competences in the clinical competence areas as 3,65 ± 0,73 (median 3,60) as "I know but not t a sufficient level". The participants 'perception of competence was "I know, but not at a sufficient level'" with an average of 3.31 ± 0.84 (median3.5) in the blood banking field, while the average in hemapheresis and transfusion medicine was 4.04 ± 0.63 (median 4) as "enough". In interventional procedures, hematologists stated that their vocational competences were 2,79± 0,92 (median 2,93) on average as "I have an idea- I know, but not enough". The correct answer to 13 theoretical questions was an average of 6,96 ± 1,89 (median 7). Hematologists performing blood rotation felt significantly more competent than the physicians who could not do the rotation in the blood bank, blood banking t(52) = -3.9, p < .001 , transfusion medicine and interventional competence t(52) = -2.2, p = .04 . Physicians who believed that they are sufficient in the blood banking area, were more confident in transfusion medicine r(54) = .67, p

Bilateral testicular tumors are very rare in pediatric patients and only a few case reports have been reported. These patients have a high risk of sterility due to bilateral orchiectomy and subsequent gonadotoxic treatments. Therefore, if possible, testis-sparing surgery should be performed in patients with benign masses and testicular tissue preservation may be recommended in order to maintain fertility in later life. We present a 23 months old boy with synchronous bilateral testicular tumor managed with unilateral orchiectomy and testis-sparing surgery and testicular tissue cryopreservation performed to the controlateral side. We also review the literature on bilateral testis tumors in children.

Children with cancer have an increased risk for hepatitis B virus (HBV) infections due to chemotherapy-induced secondary immunodeficiency and frequent blood transfusions. The aim of this study is to evaluate the efficacy and safety of hepatitis B vaccination during the intensive induction chemotherapy in children with cancer found to be seronegative for hepatitis B on admission.Children newly diagnosed with cancer were evaluated for the presence of hepatitis B surface antigen (HBsAg) and antibody on admission. The children negative for both were included in the study. A super-accelerated vaccination scheme (3 booster doses at days 1-5, 8-12, and 28-33) was administered to these seronegative children concurrently with induction chemotherapy. Antibody response was checked 4-8 weeks after the last vaccination and 6 months after the end of the treatment.Eleven out of 122 children were seronegative for hepatitis B on admission (9%). Acute lymphoblastic leukemia, lymphoma, and solid tumors were diagnosed in 5, 4, and 2 children, respectively. Complete seroconversion was achieved in 4-8 weeks after the last vaccination with high titers of anti-HBs antibody, and all patients remained antibody-positive until 6 months after the completion of chemotherapy.The risk of transfusion-related infections increases with a number of transfused products and donor exposures, and it is more significant for immunosuppressed children with hematologic and oncologic malignancies. Hepatitis B vaccination could safely be applied with brisk and sustained responses in this vulnerable population, based on the local epidemiological data.

Objective: Mastocytosis is a disease characterized by the accumulation of mast cells in one or more organ systems. The skin is the most commonly affected organ. In this study, it is aimed to present the clinical findings and follow-up of pediatric patients with cutaneous mastocytosis. Material and Methods: Between January 2015 and June 2019, 27 cases between the ages of 0-18 who were diagnosed as mastocytosis by histopathological evaluation were included in the study. Age, gender, duration of rash, disease course and additional complaints were recorded, drug, food allergy, and anaphylaxis were questioned. Results: Cutaneous mastocytosis with limited skin rash was observed in all cases. Maculopapular cutaneous mastocytosis and solitary mastocytoma were seen in 24 and 3 cases, respectively. The median age at diagnosis was 11,5 months (3 months-12 years); the rash occurred in 26 patients under 1 year of age. Three patients had a rash from birth. In addition to the rash, there were pruritus in 11 cases, atopic dermatitis in 6 cases, asthma/reactive airway in 2 cases, recurrent urticaria and flashing attacks in 1 case each. None of the patients had pathological lymphadenopathy, splenomegaly or abnormal findings on complete blood count, and eosinophilia. The cases were monitored without treatment due to the absence of systemic involvement; there was a significant decrease in the number of rashes in 6 cases, but none of the lesions completely regressed in the follow-up period. Lesions of 3 cases with solitary mastocytoma disappeared with local treatment. Symptom control was achieved in all cases with local and systemic treatment during episodes. Anaphylaxis or progression of systemic mastocytosis wasn't observed during the follow-up period. Conclusion: Our findings support the knowledge that pediatric mastocytosis is limited to skin findings and rarely progresses to systemic mastocytosis. Copyright © 2020 by Türkiye Klinikleri. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Purpose: The objective of this study is to evaluate prognostic implications of clinical, histopathological features and immuhistochemical expressions of p53, ERBB2, c-Kit and Bcl-2 in pediatric medulloblastoma. Materials and Methods: A total of 29 pediatric medulloblastoma cases were evaluated for prognostic association of demographic, clinical, histopathological features and immunohistochemical expressions of p53, ERBB2, c-Kit and Bcl-2. Results: Median age at diagnosis was 69 months (21-122 months). Median duration of follow-up was 54 months (2-209 months). Fourteen of samples were diagnosed as classical (48%), thirteen cases as nodular/desmoplastic (45%) and two cases as anaplastic (7%) subtype. Staining for c-Kit, Bcl-2, p53 and ERBB2 was positive in 28, 10, 9 and 2 samples, respectively. Overall (OS) and event-free survival (EFS) were 62 % and 52%, respectively. Bcl-2 expression was found to be significantly increased in nodular/desmoplastic subtype. None of the clinical, histopathological and immunohistochemical features were related to survival. Conclusion: This study reflects the earliest periods of current multimodal treatment protocols of medulloblastoma with similar survival rates in literature. Although none of the proposed factors have been associated with survival, future studies combining molecular and immunohistochemical methods would be more convenient for detecting new prognostic criteria in pediatric medulloblastoma.

WOS:000614296900001 PMID: 33538100 Adults with cancer are reported to have a higher risk for coronavirus disease (COVID-19) infection and more severe disease and mortality than the general population.1,2 Although children seem to be at a lower risk for COVID-19 than adults,3–5 data specifically addressing children with cancer are limited

BACKGROUND Infantile hemangiomas (IH) represent the most common type of benign tumors of infancy. Vascular endothelial growth factor (VEGF) and basic fibroblastic growth factor (bFGF) have a central role in the pathogenesis of infantile hemangiomas. METHODS In this prospective study, we aimed to investigate the relationship between serum VEGF and bFGF levels and clinical characteristics and the serological changes in VEGF and bFGF levels associated with propranolol treatment in infants diagnosed with IH. Blood samples were taken from 34 patients with IH and 10 controls. Serum VEGF and bFGF levels were studied by ELISA. RESULTS At initial diagnosis, median serum bFGF levels were 11.1 ng/ml (4.8-16.6) in patients with IH (n=34) and 2.6 ng/ml (1.7-4.7) in controls (p < 0.001), and, median serum VEGF levels for same groups were 58.5 ng/ml (25.3-190.2) and 11.4 ng/ml (8.2-19.8) (p < 0.001), respectively. Serum VEGF and bFGF levels were not correlated. In 18 infants who were treated with propranolol with serial measurements, median serum bFGF levels were 10.7 ng/ml, 9.8 ng/ml and 10.5 ng/ml (p= 0.8), and median serum VEGF levels were 68.6 ng/ml, 63.5 ng/ml and 45.1 ng/ml (p < 0.001) at initial diagnosis, at first and third months, respectively. Median regression rates of the hemangiomas at the first and third months were -%47.3 and -%58.3 (p < 0.001), respectively. CONCLUSIONS Serum bFGF levels didn`t change in time. Serum VEGF levels seemed to follow the natural course of IH and might be a marker for follow-up. The contribution of propranolol treatment should also be considered.

Bu çalışmada çocuk onkoloji servisinde solid organ malignitesi tanısı ile takip edilmiş olan hastaların kemoterapi öncesi ve sonrası hepatit b virusu (HBV) ve varisella zoster virusu (VZV) serolojilerinin değerlendirilme oranları, antikor yanıtlarının tedavi sonrası değişip değişmediğinin saptanması ve buna neden olabilecek faktörlerin araştırılması amaçlanmıştır. Çalışmamızda Mart 2016-Nisan 2019 tarihleri arasında çocuk onkoloji servisinde solid organ malignitesi tanısı ile takip edilen ve kemoterapi almış olan hastaların dosyaları geriye dönük olarak değerlendirilmiştir. Çocukluk çağı kanseri (lösemi dışı) nedeni ile takip edilmiş olan 75 hastanın 24’ü (%32) kız idi. Anti Hbs antikoru bakılan 70 hastanın 44’ünde (%58.6) anti Hbs pozitif, anti VZV antikoru bakılan 62 hastadan, 48’inde (%64) anti VZV pozitif idi. Kemoterapi öncesi anti Hbs pozitif olan 44 hastanın 8’inde (%18.1) kemoterapi sonrası anti Hbs negatifleşmiştir. Kemoterapi öncesi anti VZV pozitif saptanan 48 hastanın 4’ünde (%8.3) kemoterapi sonrası anti VZV negatifleşmiştir. Tek doz suçiçeği aşı yanıtının kemoterapi sonrası kaybolma oranı, doğal yol ile gelişmiş antikor yanıtının negatifleşme oranından daha yüksek saptanmıştır. Daha önce bu iki durumu karşılaştıran çalışma olmadığı için verilerimiz yol gösterici olabilir. Pürin analoğu, siklofosfamid, steroid kullanımı ve hastalık evresi ile anti HBs ve anti VZV serolojilerinin negatifleşmesi arasında ilişki saptanmamıştır. Onkoloji hastalarının tedavi öncesi ve sonrası bağışıklık durumlarının değerlendirilmesi konusunda daha geniş çaplı çalışmalara ihtiyaç vardır ve bu hastalar için rehberler oluşturulması uygun olacaktır., The aim of this study was to determine the rates of hepatitis b virus (HBV) and varicella zooster virus (VZV) serology assesment before and after chemotherapy, antibody response changes after therapy, and factors affecting this situation. In our study we evaluated the files of patients who were followed up with malignancy in the pediatric oncology department between March 2016 and April 2019 retrospectively. Twenty four (32%) of the 75 patients followed for childhood cancer other than leukemia were girls. Anti Hbs antibody was evaluated in 70 patients and 44 (58.6%) were positive, anti VZV antibody was evaluted in 62 patients and 48 (64%) was positive. Of the 44 patients who had anti Hbs seropositivity before chemotherapy, 8 (18.1%) were seronegative after therapy. Anti VZV was negative after therapy in 4 (8.3%) of 48 patients who had anti VZV seropositivity before chemotherapy. The rate of disappearance of vaccine response after a single dose varicella vaccine was found to be higher than the rate of negative response of naturally developed varicella antibody response. Since there were no studies comparing these two situations before, this data may be a guide. There was no correlation between purine analogue, cyclophosphamide, steroid use, disease stage and disappearance of anti Hbs and VZV antibodies. Further studies are needed to evaluate the pre-and post-treatment immune status of oncology patients, and guidelines should be developed for these patients.

BACKGROUND This study aims to evaluate the etiology of cervical lymphadenopathies in children and to define the significance of demographic, clinical, and laboratory features in the prediction of malignancy. METHODS Medical records of 527 patients were reviewed retrospectively between 2015 and 2019. The patients were examined in terms of demographics, clinical, radiologic, and serologic findings. A lymph node biopsy was performed in selected patients. The risk factors for malignancy were evaluated. RESULTS Out of 527 children, 26 had neck masses mimicking lymphadenopathy; 501 had lymphadenopathy. The most common location was the anterior cervical region and the median age was 5.7 years. Thirty-nine patients had malignancy (lymphoma in 34, nasopharyngeal carcinoma in 3, leukemia in 1 and neuroblastoma in 1). The risk of malignancy was associated with older age, duration of > 4 weeks, lymph node size > 3 cm, supraclavicular location, presence of systemic symptoms, and hepatosplenomegaly (p < 0.001, p < 0.001, p < 0.001, p < 0.001, p < 0.001, p < 0.001). On laboratory evaluation, anemia, leukocytosis, and increased erythrocyte sedimentation rate were found to be associated with malignancy (p < 0.001, p=0.003, p < 0.001). CONCLUSIONS Cervical lymphadenopathies in children are generally benign but patients with persisting cervical lymphadenopathy, adolescent age, accompanying systemic symptoms and abnormal laboratory findings should be considered for an early biopsy.

Neuroblastoma of the iris is an extremely rare clinical entity. An otherwise healthy 2-month-old male infant presented to the oncology clinic with a nodular whitish iris lesion in his right eye. The excisional tumor biopsy was consistent with a pathological diagnosis of neuroblastoma with differentiation and negative MYCN gene mutation. Further systemic evaluation revealed a right adrenal mass with no metastatic lesion. The biopsy of the adrenal lesion was also consistent with neuroblastoma. After four courses of chemotherapy, the adrenal mass was completely resected. The patient underwent two additional courses of postoperative chemotherapy and continued retinoic acid treatment. The patient is under regular follow-up with no evidence of recurrence 36 months after the initial diagnosis. This is the first case report to present a histopathological verification of neuroblastoma of the iris. The authors suggest that neonates and infants who are diagnosed as having neuroblastoma undergo an ophthalmologic examination after the initial diagnosis to investigate the true incidence of small iris lesions in neuroblastoma that may have been unrecognized. Neuroblastoma should be included in the differential diagnosis of amelanotic iris lesions in infants and young children. [ J Pediatr Ophthalmol Strabismus . 2019;56:e12–e16.]

Background Kaposi`s sarcoma (KS) is a complication of immunosuppressive therapy for transplant recipients. Unlike adult recipients, KS in pediatric organ transplantation is quite rare. Treatment is usually withdrawal of immunosuppression; non-responders often receive chemotherapy. Case We have reported a child with post-liver transplant visceral KS which has progressed despite withdrawal of immunosuppressive therapy, who has been treated with Paclitaxel for three weeks. KS has regressed completely after four cycles of Paclitaxel. Conclusion Paclitaxel should be considered as an effective first line treatment option for patients with posttransplant KS.

Summary Aim: The aim of the study was to determine the differences between rural and urban adolescents rsquo; health and health behaviors Material and Method: The data presented in this study were a part of the Turkish data nbsp; collected for the cross sectional and international nbsp; ldquo;Health Behavior in School aged Children 2005 2006 study rdquo; which aimed to evaluate adolescents rsquo; health and health behavior around Europe and North nbsp; America Data were collected from 3884 11 to 13 years of age nbsp; 1946 males 1938 females students who attended nbsp; 180 classes of 82 primary schools in nbsp; 23 cities Statistical analyses were made by using chi square nbsp; test Results: Both female and male students who lived in rural areas were more likely to go to bed hungry more often to feel dizzy more often to eat nbsp; fruits less often to brush teeth less often to exercise less often and to use computers less often than their peers living in urban areas Besides in rural areas nbsp; male students were more likely to be violent and female students were more likely to feel unhealthy to be unsatisfied with their lives and to be injured more often than their urban peers Both female and male students who lived in urban areas were more likely to eat or drink more energy dense and low nutritional value foods and drinks than their peers living in rural areas Males who lived in urban areas were more likely to be on a diet not to be satisfied with their body and to be obese than their peers living in rural areas nbsp; Conclusions: The results of this study showed that there are several health inequities between rural and urban female and male adolescents These differences should be taken into account when prepering adolescent health promotion programs Turk Arch Ped 2010; 45: 96 104 Key words: Adolescent health health behavior rural urban differencest, Özet Amaç: Bu araştırmanın amacı Türkiye rsquo;de kentsel ve kırsal alanlarda yaşayan 11 ve 13 yaşındaki öğrencilerin sağlık durumları ve sağlıkla ilgili davranışları arasındaki farklılıkları saptamaktı Gereç ve Yöntem: Bu çalışmada gençlerin sağlık durumları ve sağlık davranışlarını saptamayı hedefleyen kesitsel tanımlayıcı ve çok uluslu ldquo;Health Behavior in School Aged Children 2006 HBSC rdquo; araştırmasının Türkiye ayağına katılan 11ve 13 yaşındaki nbsp; çocukların verilerinden yararlanıldı Bu çalışma için veri çözümlemeleri 23 ildeki 82 ilkokulun 180 dersliğinde 11 ve 13 yaşlarındaki 3884 1946 erkek 1938 kız öğrenci üzerinden yapıldı Gruplar arasındaki oran farklılıkları ki kare testi ile değerlendirildi Bulgular: Kırsal alanda yaşayan hem kızların hem de erkeklerin kentlerde yaşayan akranlarından daha sık yatağa aç girdikleri daha sık baş dönmesinden yakındıkları daha az meyve yedikleri daha nadir diş fırçaladıkları daha az egzersiz yaptıkları saptandı Kırsal alanda yaşayan öğrenciler kentlerde yaşayanlara göre daha az bilgisayar kullanıyorlardı Ayrıca kırsal alanda yaşayan erkeklerin daha sık şiddet davranışları gösterdikleri ve kızların ise kendilerini daha sağlıksız hissettikleri yaşamlarından daha az memnun oldukları ve daha sık yaralandıkları saptandı Kentlerde yaşayan nbsp; kız ve erkek öğrenciler kırsal alanda yaşayan akranlarından daha sık olarak besin değeri düşük ve enerji içeriği yüksek yiyecek ve içecekleri tükettiklerini bildirdiler Kentsel alanda yaşayan erkekler arasında diyette olma bedeninden hoşnut olmama ve şişmanlık kırsal alanda yaşayanlara göre daha sıktı Çıkarımlar: Araştırmamız ülkemizde kentsel ve kırsal alanda yaşayan 11 ve 13 yaşındaki kızlar ve erkekler arasında sağlık açısından eşitsizlikler olduğunu ve kırsal alanda yaşayan hem kızların hem de erkeklerin daha fazla risk altında olduğunu göstermektedir Ergenler için sağlığı geliştirme programları hazırlanırken yerleşim yeri nedeni ile ortaya çıkan bu farklılıkların göz önüne alınması gerektiğini düşünmekteyiz nbsp; Türk Ped Arş 2010; 45: 96 104 Anahtar sözcükler: Ergen kırsal kentsel farklılıklar sağlık sağlık davranışları

Amaç: Bu araflt›rman›n amac› Türkiye’de okula devam etmekte olan ergenlerin fiziksel etkinlik düzeylerinin demografik etkenler, beden kitle indeksi ve baz› sa¤l›k davran›fllar› ile iliflkisini saptamakt›. Gereç ve Yöntem: Bu çal›flmada kesitsel tan›mlay›c› ve çok uluslu “Health Behavior in School Aged Children (HBSC)” araflt›rmas›n›n Türkiye aya¤›na kat›lan ve 6., 8. ve 10. s›n›fa devam eden 11, 13 ve 15 yafl›ndaki ergenlerin verilerinin bir bölümü de¤erlendirildi. Veri çözümlemeleri 26 ildeki 82 ilkokulun 180 dersli¤inde 11 ve 13 yafllar›ndaki 3884 (1946 erkek, 1938 k›z) ve 83 lisenin 88 dersli¤inde 15 yafl›ndaki 1668 (901 erkek, 767 k›z) ö¤renci üzerinden yap›ld›. ‹statistiksel de¤erlendirmede ki-kare testi, faktör analizi ve lojistik regresyon analizi kullan›ld›. Bulgular: Ö¤rencilerin %20’si haftada en az yedi gün, günde en az bir saat herhangi bir fiziksel etkinlikte bulunduklar›n› bildirdi. Çok de¤iflkenli analizde (lojistik regresyon) fiziksel etkinlik ile erkek olmak, 15 yafl›na göre 11 yafl›nda ve 13 yafl›nda olmak ve akflamlar› arkadafllar› ile evin d›fl›nda zaman geçirmek aras›nda anlaml› iliflki bulundu. Beslenme davran›fllar› hem sa¤l›kl› hem de sa¤l›ks›z yiyecekleri az tüketenler esas al›narak incelendi¤inde, di¤er üç grubun (sa¤l›kl› yiyecek tüketimi s›k+sa¤l›ks›z yiyecek tüketimi seyrek olanlar; sa¤l›ks›z yiyecek tüketimi s›k+sa¤l›kl› yiyecek tüketimi seyrek olanlar; her iki tip yiyece¤i s›k tüketenler) esas gruba göre fiziksel etkinlik düzeylerinin daha fazla oldu¤u saptand›. Çıkarımlar: Bu araflt›rman›n sonuçlar› ülkemizde yaflayan ergenlerin fiziksel etkinlik düzeylerinin yetersiz oldu¤unu, özellikle k›zlar›n fiziksel olarak etkin olmad›¤›n›, ergenlerin beslenme al›flkanl›klar›n›n ve arkadafllar› ile geçirdikleri zaman›n fiziksel etkinlik düzeyleriyle iliflkili oldu¤unu göstermesi aç›s›ndan önemlidir. Etkin sa¤l›¤› koruma ve gelifltirme programlar› özellikle k›zlara odaklamal› ve ergenlerin beslenme al›flkanl›klar›n›n üzerinde durmal›d›r. Akranlar›n ergenlerin fiziksel etkinlik düzeylerinin art›r›lmas›nda önemli rol oynayabilecekleri de unutulmamal›d›r., Aim: The aim of this study was to determine the relationships between physical activity level and demographic characteristics nbsp; body mass index nbsp; and some other health behaviors nbsp; in Turkish adolescents

The aim of this study was to explore the relationships between involvement in bullying behaviors and school, family, and peer factors. Health Behavior in School Age Children survey questionnaire was used. Of the students surveyed, 20% were both bully and victim, 11% were bully, and 21% were victim. Being male, poor parental support, and poor monitoring by the father were found to be risk factors for being both bully and victim. Poor academic achievement, having peers at different ages, poor quality of friendship, poor communication with parents, and not being isolated by peers were found to be risk factors for being bully. Not liking school, feeling pressured by school work, poor quality of friendship, poor monitoring by the father, close bonding with mother, and poor status of the peer group were found to be risk factors for being victim. These findings highlight the importance that bullying intervention programs should include country-specific and culture-specific influences for success.

Takayasu's arteritis is a chronic inflammatory disease that primarily involves the aorta and its main branches. Varying degrees of narrowing, occlusion, or dilatation develop in the involved vessel segments. However, dissection of the aorta is quite rare in this disease, and it may develop particularly after angioplasty. We report a very rare case of Takayasu's arteritis with dissection of the abdominal aorta just distal to the origin of the inferior mesenteric artery in a 9-year-old girl. She was treated conservatively with close follow-up. At the end of 1 year's follow-up, the dissection of the aorta did not show progression, and new lesions were not identified. To our knowledge, this patient is the youngest child presented with arterial dissection as the initial manifestation of the disease.

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.

Nicolau syndrome (livedoid dermatitis) is a very rare complication of intramuscular injections and manifests as excruciating pain immediately after injection. We describe a 3-year-old boy with diagnosis of Nicolau syndrome after intramuscular benzathine penicillin injection to the midanterior part of the left thigh. He was treated with hyberbaric oxygen and pentoxyphilline in addition to supportive treatment and recovered with no sequelae.

The aim of this study was to evaluate the incidence of anemia detected in familial Mediterranean fever (FMF) and the effect of disease activity and colchicine therapy along with interleukins to laboratory tests, including serum transferrin receptor (TfR), in the diagnosis of anemia seen in FMF in children. After detecting anemia in 63.4% of 172 FMF patients followed up by our rheumatology outpatient polyclinics, it was decided to study 3 groups of patients: group 1, 17 newly diagnosed FMF patients; group 2, 36 FMF patients on colchicine therapy; and group 3, 17 healthy children as control for the symptom of anemia. All 3 groups of patients were investigated for their hematological parameters, iron status, including soluble transferrin receptor (sTFR) concentrations and sTFR index, and IL-6 levels. Anemia ratio was 9/17, 53%; 11/36, 31%; and 1/17, 5% in the groups 1, 2 and 3, respectively. There was a significant difference between hemoglobin (Hb) values in the first group and the second (patients who were on colchicine therapy). Furthermore, in the second group there was a significant difference between the Hb concentrations at the time of diagnosis and after colchicine therapy (p = .003). There was a positive correlation between Hb and plasma iron and transferrin saturation in group 1 and disease beginning age, iron, transferrin saturation, and erythrocyte sedimentation rate (ESR) in the second group. In the first group the anemic patients' iron and transferrin saturation were significantly lower than normal, while ferritin levels were higher. In the second group, a good correlation was found with ESR and Hb levels; the higher ESR values were detected in patients with lower Hb values. Of the anemic and nonanemic patients of the first and second groups, values for interleukin 6 and iron parameters, including sTFR, were found similar. Anemia detected in FMF patients was found related to iron status more than interleukins. Colchicine therapy had a positive effect on anemia as well as on disease activity. Resolution of symptoms of FMF occurred with correction of the anemia, if the patient ESR values also decreased on colchicine therapy.

Spinal muscular atrophy (SMA) is an autosomal recessive disorer characterized by progressive loss of motor neurons in the spinal ord and lower brainstem. Patients are classified into three types SMA I–III), based on the age of onset and clinical severity. SMA ype I is the most severe form in which symmetric muscle weakness evelops in the trunk and proximal parts of the extremities within months of birth. More than 90% of all patients show homozyous deletion of survival motor neuron 1 gene (SMN1), which is ocated on human chromosome 5. Low levels of SMN protein results n degeneration of motor neurons and extraneuronal manifestaions of disease such as cardiac defects. Atrial or ventricular septal efects and defects associated with left ventricular outflow tract bstruction were reported in patients with SMA [1]. In this report, e describe an infant with tetralogy of Fallot and type 1 spinal uscular atrophy.

Sickle cell anemia SCA is the most common hemoglobinopathy all over the world. While the overall incidence of sickle cell disease trait in Turkey is %0.3-0.6, especially some places in Çukurova area it reaches the level of 3-44%. Painful vaso- occlusive crisis which is the most important complication of this disease, is predicted to increase in frequency and HbS polymerization is responsible in its pathophysiology. As an acute complication of SCA, acute chest syndrome is the second most common cause of hospitalization and mortality. İnitial approach to patients with an acute chest syndrome includes supportive treatments. Whereas, solely transfusion is not enough for treatment if there is worsening in respiratory signs and chest radiography; deepening of hypoxemia which requires an oxygen support. Therapeutic red cell apheresis can be used with the aim to treat acute complication of sickle cell disease. In this article we present successful therapeutic red cell apheresis and supportive treatments that we practise in our case who developed acute chest syndrome, and followed up by us with the diagnosis of sickle cell disease, Orak hücre anemisi OHA dünyada en sık görülen hemoglobinopatilerdendir. OHA taşıyıcılığı sıklığı Türkiye genelinde %0.3-0.6 arasındayken, özellikle Çukurova bölgesinde bazı yörelerde bu sıklık %3-44’e ulaşmaktadır. Sıklığının artması öngörülen bu hastalığın en önemli komplikasyonu vazookluzif ağrılı krizler olup patofizyolojisinde HbS polimerizasyonu sorumludur. Akut komplikasyonlar arasında gösterilen akut göğüs sendromu; ikinci en sık hastanede yatış ve mortalite nedeni olarak gösterilmektedir. Akut göğüs sendromu gelişen hastalara ilk yaklaşım destekleyici tedaviler iken, solunum bulgularında ve akciğer grafisinde kötüleşme, oksijen desteği gerektirecek kadar hipokseminin derinleşmesi durumunda; basit transfüzyon tedavi için yeterli olamamaktadır. Terapötik eritrosit aferezi; orak hücre hastalığı komplikasyonlarını tedavi etmek amacı ile yapılabilmektedir. Bu makalede orak hücre anemili tanısı ile izlenen ve akut göğüs sendromu gelişen vakamızın, destek tedavileri ve terapötik eritrosit aferezi ile başarılı tedavisi sunulmuştur