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Amaç: Bu çalışmada, akut lenfoblastik lösemi (ALL) tanısıyla tedavi edilmiş çocuk hastaların tedavi esnasında görülen nörolojik komplikasyonların değerlendirilmesi, tanıda ve tedavide nörolojik komplikasyon görülme risk faktörlerinin saptanması ve nörolojik komplikasyonların prognoza etkisinin değerlendirilmesi amaçlanmıştır. Yöntemler: Çalışmamızda kliniğimizce Ocak 2006 ile Aralık 2011 tarihleri arasında 18 yaş altı ALL tanısı almış ve Berlin Frankfurt Münster (BFM) TR ALL-2000 kemoterapi protokolü uygulanmış olan hastalar, hastane dosya ve kayıt sisteminden geriye dönük incelenmiştir. Çalışmaya dâhil edilen 200 hastanın 6’sı ilk tanıda merkezi sinir sistemi tutulumu olması nedeniyle çalışma dışı bırakıldı. Yüz doksan dört hastanın demografik, klinik, laboratuvar, radyolojik ve nörolojik bulguları değerlendirmeye alındı. Nörolojik komplikasyon görülen olgularla görülmeyen olgular iki gruba ayrılıp cinsiyet, yaş, laboratuvar, tedavi, sağ kalım ve relaps açısından kıyaslandı. Bulgular: İlk tanıda merkezi sinir sistemi tutulumu olmayan 194 hastanın 28’inde tedavi esnasında nörolojik komplikasyon görüldü. En sık saptanan nörolojik komplikasyon konvülzyondu (%7,7). Nörolojik komplikasyon gelişen hastalarda erkeklerin çoğunlukta olduğu görülmekle birlikte, cinsiyet ve yaş ile nörolojik komplikasyon gelişimi açısından istatistiksel anlamlı ilişki saptanmadı. (p0,05) Sonuç: Çocukluk çağı ALL’sinde kötü bir prognostik faktör olduğu bilinen tanıdaki lökosit sayısının yüksekliğinin, tedavi esnasında görülebilecek nörolojik komplikasyonların habercisi olabileceği akılda tutulmalıdır.

Children with thalassemia major (TM) are prone to growth failure and micronutrient deficiency. Thus, this study aimed to evaluate nutritional status, anthropometrics, and bone mineralization defects in patients with regular blood transfusion.Data obtained were analyzed by evaluating laboratory tests, anthropometric measures, and bone mineral density.This study included 29 patients (62% male and 38% female) with a mean age of 12.26±4.74 years, mean pre-transfusion hemoglobin of 8.64±1.01 g/dL, and mean serum ferritin of 1158.6±556.8 ng/ mL. Vitamin D (72.4%), selenium (72.4%), and folate (37.9%) deficiencies were most frequent. Hypocalcemia was observed in 17.2%, hypomagnesemia in 3.5%, and decreased ceruloplasmin in 10.3% of patients. Folate was higher between 2 and 6 years old (p=0.028). Ceruloplasmin was higher between 6 and 10 years old (p=0.018). Selenium was significantly higher in patients with a ferritin of ≥1,500 (p=0.008). No significant ferritin-related differences were found in other micronutrients (p0.05). Body mass index (BMI) were5 percentile (p) in 31% of patient, whereas none was95 p. Height in 24.5% and weight in 20.7% of patients were3 p, whereas none with97 p. BMI of patients aged 10-18 years was significantly higher (p=0.001). Anthropometric percentiles did not significantly differ in the mean serum ferritin and micronutrient levels. Hypoparathyroidism was observed in 13.8% and hypothyroidism in 3.5% of patients. Low bone density was detected in 14.8% (2 osteopenic and 2 osteoporotic) of patients. Bone mineral density did not significantly differ in the ferritin and micronutrient levels.Nutritional support and deficiency prevention are important to minimize the burden of complications and increase the life expectancy and quality in patients with TM.Talasemi majör (TM) tanılı çocuklar büyüme geriliğine ve mikrobesin eksikliklerine yatkındırlar. Bu çalışmada, düzenli transfüzyon uygulanan hastalarda mikro besin seviyelerini, antropometrik ölçümleri, kemik mineralizasyon defektlerini değerlendirmeyi amaçladık.Laboratuvar testlerini, antropometrik ölçümleri ve kemik mineral yoğunluğunu değerlendirerek elde edilen verileri analiz ettik.Çalışmaya ortalama yaşı 12,26±4,74 yıl, ortalama transfüzyon öncesi hemoglobin 8,64±1,01 g/dL, ortalama serum ferritin 1158,6±556,8 ng/mL olan 29 hasta (%62’si erkek, %38’i kız) alındı. En sık vitamin D (%72,4), selenyum (%72,4) ve folat (%37,9) eksikliği saptandı. Hastaların %17,2’de hipokalsemia, %3,5 hipomagnesemi, %10,3 seruloplazmin düşüklüğü saptandı. Folat 2≤ ile6 yaş arasında, seruloplazmin 6≤ ile10 yaş arasında daha yüksek saptandı (sırasıyla p=0,028, p=0,018). Selenyum, ferritin ≥1.500 olan hastalarda anlamlı olarak yüksekti (p=0,008). Diğer mikrobesin düzeylerinde ferritin düzeyi ilişkili anlamlı farklılık saptanmadı (p0,05). Hastaların %31’inin vücut kitle indeksi (VKİ) 5. Persentilin (p) altındaydı. VKİ 95. p’nin üzerinde olan hasta saptanmadı. Hastaların %24,5’inin boyu, %20,7’sinin vücut ağırlığı 3. p’nin altındaydı. Boy ve vücut ağırlığı açısından 97. p’nin üzerinde olan hasta saptanmadı. Antropometrik ölçümlerin, hastaların kan mikrobesin ve ferritin düzeyleri ile anlamlı bir ilişkisi saptanmadı. On-18 yaş arasındaki hastaların VKİ anlamlı olarak daha yüksek bulundu (p=0,001). Antropometrik ölçümler, ortalama ferritin ve mikrobesin düzeyleri açısından anlamlı farklılık göstermedi. Hastaların %20,8’de endokrinolojik bozukluklar (hipoparatiroidi %13,8, hipotiroidi %3,5) gözlendi. Hastaların %14,8’inde (2 osteopenik, 2 osteoporotik) düşük kemik yoğunluğu saptandı. Kemik mineral yoğunluğunun mikrobesin ve ferritin düzeyleriyle anlamlı ilişkisi saptanmadı.TM hastalarında nütrisyonel eksikliklerin önlenmesi ve beslenme desteğinin sağlanması kemik mineralizasyon bozuklukları ve büyüme geriliği gibi komplikasyonların gelişiminin en aza indirilmesi açısından önemlidir.

Background Regarding the difficulties in recognition and management of the malignancies in primary immune deficiencies (PIDs), we aimed to present the types, risk factors, treatment options, and prognosis of the cancers in this specific group. Methods Seventeen patients with PID who developed malignancies or malignant-like diseases were evaluated for demographics, clinical features, treatment, toxicity, and prognosis. Results The median age of malignancy was 12.2 years (range, 2.2-26). Lymphoma was the most frequent malignancy (n = 7), followed by adenocarcinoma (n = 3), squamous cell carcinoma (n = 2), cholangiocarcinoma (n = 1), Wilms tumor (n = 1), and acute myeloid leukemia (n = 1). Nonneoplastic lymphoproliferation mimicking lymphoma was observed in five patients. The total overall survival (OS) was 62.5% ± 12.1%. The OS for lymphoma was 62.2% ± 17.1% and found to be inferior to non-PID patients with lymphoma (P = 0.001). Conclusion In patients with PIDs, malignancy may occur and negatively affect the OS. The diagnosis can be challenging in the presence of nonneoplastic lymphoproliferative disease or bone marrow abnormalities. Awareness of susceptibility to malignant transformation and early diagnosis with multidisciplinary approach can save the patients' lives.

Mean platelet volume (MPV) and platelet distribution width (PDW) can help diagnose cardiovascular pathologies. In this study, we aimed to demonstrate the changes in platelet (PLT) indices in children diagnosed with bicuspid aortic valve (BAV) with mild stenosis and without stenosis to compare patients with mild stenosis with those without stenosis. A total of 73 children diagnosed with BAV (30 patients with mild stenosis and 43 without stenosis) with a mean age 9.73 ± 5.01 years and a control group were included in the study. Mean MPV value was significantly lower in the control group compared with patients with BAV with mild stenosis and patients without stenosis (p = 0.001, and p 0.01, respectively). MPV was significantly greater in patients with mild stenosis than in patients without stenosis (p = 0.049 and p 0.05, respectively). Patients with mild stenosis had a significantly greater mean PDW value compared with patients without stenosis and the control group (p = 0.024 and p 0.05, respectively). There was no significant difference between patients without stenosis and the control group with respect to mean PDW value (p 0.05). In conclusion, the results of this study demonsrate that children with BAV either with or without stenosis have increased MPV; the ones with mild stenosis have even greater values than the ones without stenosis. It emphasizes the risk of thrombosis in children with BAV.

Candida enfeksiyonu myokardit, endokardit ve dilate kardiyomyopatinin nadir bir sebebidir. Antifungal tedavinin baslanma zamanin bu hastalarda mortalite oranlarini etkiledigi iyi bilinmektedir. Kardiyak Candida enfeksiyonlarindaki optimal antifungal secenegi ve tedavi suresi ise henuz kesin olarak tanimlanmamistir. Bu calismada akut lenfoblastik losemi tanili dort yasindaki bir cocukta Candida albicansa bagli nadir bir dilate kardiyomiyopati ve endokardit vakasi paylasilarak tedavisi tartisildi. (J Pediatr Inf 2014; 8: 82-5) Anahtar kelimeler: Dilate kardiyomiyopati, candida, akut losemi Abstract

Trombosis is seen in children with acute lymphoblastic leukemia during or after L-asparaginase treatment. Posterior reversible encephalopathy syndrome (PRES) is a complex syndrome characterized with sudden hypertension, headache, nausea, vomiting, alteration in the state of consciousness, vision defect and seizures. The cases related to this syndrome have been reportedly seen after eclampsia, organ transplantation, immunsuppressive treatments, autoimmune diseases and chemotherapy. Vasogenic edema occuring in the brain parencyhma constitues the basic pathophysiology. We present a case who developed seizures during treatment for B-cell acute lymphoblastic leukemia and diagnosed as posterior reversible encephalopathy.

Amac: Bu calismanin amaci cocuk Hodgkin hastaliginda hastalarin yasam suresi ve tedaviye yanitini inceleyerek sagkalimi etkileyen degiskenleri arastirmaktir Gerec ve Yontem: Hastanemiz Cocuk Hematoloji Onkoloji Klinigi rsquo;nde 1996 2011 yillari arasinda izlenen ve tedavi edilen 43 Hodgkin lenfoma hastasi geriye donuk olarak incelendi On sekiz yas altinda yeni tani almis 23 erkek ve 20 kiz hasta calismaya dahil edildi Bulgular: Ortanca yas dokuz yil bes ay olup bir hasta evre I 21 hasta evre II 12 hasta evre III ve dokuz hasta evre IV idi Hastalar erken evre ve ileri evre olarak siniflandirildi Evre I evre IIA ve dusuk riskli evre IIB hastalar erken evre evre III evre IV ve yuksek riskli evre IIB hastalar ileri evre olarak degerlendirildi On dokuz hasta erken evre 24 hasta ileri evre idi En sik histolojik alt tip 22 hasta ile noduler sklerozan tipti On sekiz hastada B bulgulari vardi Calismada hemoglobin duzeyi lokosit sayisi eritrosit cokme hizi bakir fibrinojen laktik dehidrojenaz ferritin ve haptoglobin duzeylerinin tedaviye yaniti uzerine etkisi arastirildi Otuz sekiz hasta yasamakta olup yedi hastada hastalik yineledi ve bes hasta oldu Ortanca izlem suresi yedi yil dort aydi Ortalama sagkalim suresi 12 66 plusmn;0 70 yildir En son olum besinci yilda gorulmus olup; bu aydaki kumulatif sagkalim orani 85 9 standart hata 6 1 rsquo;dir Erken evrelerde ortalama sagkalim suresi 13 10 plusmn;0 81 yil ileri evreler icin 12 56 plusmn;0 96 yildi Cikarimlar: Bulky hastalik ortalama sagkalim suresi icin kotu seyir degiskeniydi Kadin cinsiyet bulky hastalik ve noduler sklerozan alt tipi hastaliksiz yasam icin kotu seyir etmeniydi Turk Ped Arfl 2013; 48: 310 4

In this study, we aimed to determine the frequency of pericardial effusion (PE) in children diagnosed with acute lymphoblastic leukemia (ALL). Clinical features of patients with effusion were evaluated. For this purpose, we reviewed the medical records of ALL patients who had pretherapy echocardiograms. A total of 90 patients aged between 1.8 and 16.3 years were analyzed retrospectively. In 23 of 90 (25.6%) patients, PE was detected at initial diagnosis. The age of patients with PE ranged between 1.8 and 14.8 years (mean 5.05 ± 3.77 years). The female/male ratio was 9/14. Six (26.1%) patients were T-lineage and 17 (73.9%) were B-lineage ALL. Nine (39%) patients were in standard risk group, 13 (57%) were in median risk group, 1 (4%) patient was in high-risk group. Mean initial white blood cell count was 40.756 ± 38.653/mm(3) (range 23.000-130.000/mm(3)). Mean initial hemoglobin count was 7.3 ± 1.39 gr/dL (range 5.5-10.1 gr/dL), mean initial platelet count was 35.200 ± 26.300/mm(3) (range 4.000-118.000 mm(3)). Size of effusions was between 2 and 6 mm (mean size 3.3 ± 1.8 mm). All patients had normal left ventricular systolic function. In 87% of patients, effusions disappeared in the first 7 days and, in 13%, disappeared between 8th and 15th days of chemotherapy. None of the patients required pericardiocentesis. Cardiac dysfunction did not occur among any of these patients during chemotherapy. In conclusion, PE is not frequent in childhood ALL. It usually does not cause cardiac impairment. It responds to treatment of leukemia.

This study aimed to assess the demographic data and treatment results of children who were diagnosed with Burkitt lymphoma and treated according to the Berlin-Frankfurt-Münster-95 (BFM) protocol in a single institution. A total of 48 patients (37 boys, 77%) with a median age of 8 years (range 2 to 16 years) at diagnosis, were evaluated. Primary tumor sites were abdomen (70.8%), head and neck (22.9%), peripheral lymph node (2%), bone (2%), and testis (2%). The 5-year overall survival (OS) and event-free survival (EFS) were 78.1±4% and 76.6±6%, respectively. In univariate analysis, hemoglobin level less than 10 g/dL, cerebrospinal fluid (CSF) positivity and dialysis requirement at diagnosis were found to be important reverse predictor factors for EFS (P; 0.001, 0.001, 0.004, respectively). In multivariate analysis, hemoglobin level less than 10 g/dL and dialysis at diagnosis were found to be important reverse predictor factors for EFS (P; 0.0001). The EFS of our patients was lower than the values achieved with BFM-95 protocol in other centers. This study provides evidence that low hemoglobin level, CSF positivity and dialysis at diagnosis were important predictor factors for EFS in children with Burkitt lymphoma.

Yörük MA, Erat-Nergiz M, Timur Ç, Canbolat-Ayhan A, Ergüven M. Chylous ascites after lymphadenectomy in a Wilms` tumor patient. Turk J Pediatr 2018; 60: 436-438. Wilms` tumor is the most common renal malignancy in children and the fourth most common childhood cancer. It accounts 6-7% of all childhood malignancies. Surgical resection is an important therapy option and transabdominal or transperitoneal resection with lymph node sampling is preferred. Development of chylous ascites following intraabdominal or retroperitoneal resection in pediatric age group generally results from extensive lymph node dissection, accidental ligation or interruption of lymphatic ducts. Diseases or conditions affecting abdominal and/or retroperitoneal lymph nodes may cause chylous ascites. Postoperative chylous ascites is associated with significant morbidity and may cause mechanic, nutritional and immunological complications. In the present study, a 16-month-old infant with Stage IV Wilms` tumor who developed chylous ascites after left nephrectomy and lymphadenectomy will be presented; chylous ascites treatment with enteral nutrition and surgical treatment approach for Wilms` tumor will be reviewed.

Immune thrombocytopenia (ITP) is an acute self-limited disease of childhood, mostly resolving within 6 months irrespective of whether therapy is given or not. Treatment options when indicated include corticosteroids, intravenous immune globulin (IVIG), and anti-RhD immunoglobulin. We reviewed our 32 years' experience for first-line therapy of acute ITP. Five hundred forty-one children (mean age: 5.3 years) diagnosed and treated for ITP were evaluated retrospectively. Among 491 acute ITP patients, IVIG was used in 27%, high-dose steroids in 27%, low-dose steroids in 20%, anti-D immunoglobulin G (IgG) in 2%, and no therapy in 22%. When the initial response (platelets >50 × 10(9)/L) to first-line treatment modalities were compared, 89%, 84%, and 78% patients treated by low-dose steroids, high-dose steroids, and IVIG responded to treatment, respectively (P > .05). Mean time to recovery of platelets was 16.8, 3.8, and 3.0 days in patients treated with low-dose steroids, high-dose steroids, and IVIG, respectively (P < .0001). Thrombocytopenia recurred in 23% of low-dose steroid, 39% of high-dose steroid, and in 36% of IVIG (P < .0001) treatment groups. Of 108 patients who were observed alone, 4 (3%) had a recurrence on follow-up and only 2 of these required treatment subsequently. Recurrence was significantly less in no therapy group compared with children treated with 1 of the 3 options of pharmacotherapy (P < .0001). Response rates were similar between patients treated by IVIG and low- and high-dose steroids; however, time to response was slower in patients treated with low-dose steroids compared with IVIG and high-dose steroids.

Aim: The aim of this study is to analyze the response and survival rate of pediatric Hodgkin-s disease and investigate the parameters influencing the survival.

Hypereosinophilic syndrome (HES) and the association of hypereosinophilia with acute lymphoblastic leukaemia (ALL) are both rare in children. Some acute myelogenous leukaemias can present with eosinophilia, but the relationship between HES and ALL is not well known and is rarer than the relationship between HES and acute myelogenous leukaemia. Patients are diagnosed with HES when no cause is found to explain the eosinophilia leading to end organ damage. For this reason, it is recommended that patients presenting with hypereosinophilia be carefully assessed to exclude any malignant clonal proliferation. HES may present with severe clinical manifestations such as high leucocyte count, anaemia, thrombocytopaenia, hepatosplenomegaly or cardiac and neurological involvement, all of which are primarily features of myeloproliferative disorders. Some patients with HES can develop chronic eosinophilic leukaemia. Successful treatment of HES with agents used in chronic myeloid leukaemia supports the idea that HES can be a chronic myeloid disorder. There are few cases reporting an association between ALL and hypereosinophilia that precedes or is concomitant with ALL. Here we report the case of a 14-year-old girl who developed common B ALL 7 months after diagnosis and treatment of HES. Interestingly, eosinophilia was not concomitant with the diagnosis of ALL.

Background It is well known that survivors of acute lymphoblastic leukemia (ALL) show a tendency to become overweight. Cranial irradiation (CRT), is considered to be the primary risk factor for development of obesity. Aims The aim of our study was to evaluate body mass index (BMI) and serum leptin levels in survivors of childhood ALL. Methods Subjects (Group I) consisted of 93 survivors of childhood ALL (53 males > or = 9 years old, 40 females > or = 8 years old) diagnosed between January 1975 and December 2002 in the Hematology-Oncology Division in Cerrahpasa Medical Faculty, Istanbul University after a follow up 10.21 +/- 4.90 (mean +/- SD) years. Fifty healthy individuals of similar chronological age were taken as controls (29 males, 21 females). Seventy-four subjects had received radiotherapy (Group IA) and 19 had not (Group I B). Results In Group I, BMI was significantly higher than in Group II (21.65 +/- 4.02 vs 20.31 +/- 3.49, p = 0.04). However, BMI was significantly higher only in Group I A (21.83 +/- 4.27) than in Group II (p = 0.032). Leptin levels were significantly higher in Group I A females than in Group II females. There was a significant correlation between BMI-SDS and serum leptin levels in group IA females. Conclusions Leukemia treatment leads to obesity. Higher leptin levels in girls may suggest that sex may be a differentiating factor for this late effect.

Aspergillus can causes invasive disease of various organs especially in patients with weakened immune systems. Aspergillus synovitis and arthritis are uncommon types of involvement due to this infection. Approches to fungal osteoarticular infections are based on only case reports. This paper presents a rare case of chronic granulomatous Aspergillus synovitis in an immunocompromised 5-year old girl who was treated for acute lymphoblastic leukemia.