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In this study, we aimed to investigate changes in calcium (Ca) metabolism in hemophilia patients (PWH). We also aimed to investigate the importance of diagnosis and treatment of factors impairing calcium metabolism and the significance of early diagnosis and prophylaxis with respect to these subjects. For all patients, serum calcium, phosphorus, alkaline phosphatase, 25 hydroxy vitamin D (25-OHD), parathormone (PTH), and calcitonin levels were evaluated. Bone mineral density (BMD) was assessed by dual-energy x-ray absorptiometry. Low BMD scores and 25-OHD deficiency were observed in 29 (74.4%) and 34 (87.2%) patients, respectively. Prophylaxis of PWH did not differ significantly in terms of 25-OHD levels and BMD scores. Patients in the prophylaxis group had significantly higher PTH levels (P=0.042). A negative correlation was found between PTH measurements and Z-score (P=0.008). In summary, our findings, with a small number of PWH in our study group, suggest that biochemical markers of bone turnover may be used to detect bone loss. Follow-up through annual BMD measurements coupled with appropriate exercise programs could be recommended.

Improvement in long-term survival in patients with acute lymphoblastic leukemia (ALL) in childhood has led to the need for monitorization of treatment-related morbidity and mortality. In the current study, we aimed to evaluate endocrine side effects of treatment in ALL survivors who were in remission for at least 2 years.Sixty patients diagnosed with ALL, who were in remission for at least 2 years, were cross-sectionally evaluated for long-term endocrine complications.The median age of the patients at the time of diagnosis, at the time of chemotherapy completion, and at the time of the study was 5 years (minimum-maximum: 1.7-13), 8 years (minimum-maximum: 4.25-16), and 11.7 years (minimum-maximum: 7-22), respectively, and median follow-up time was 4 years (minimum-maximum: 2-10.1). At least one complication was observed in 81.6% of patients. Vitamin D insufficiency/deficiency (46.6%), overweight/obesity (33.3%), and dyslipidemia (23.3%) were the three most frequent endocrine complications. Other complications seen in our patients were hyperparathyroidism secondary to vitamin D deficiency (15%), insulin resistance (11.7%), hypertension (8.3%), short stature (6.7%), thyroid function abnormality (5%), precocious puberty (3.3%), and decreased bone mineral density (1.7%). There were no statistically significant correlations between endocrine complications and age, sex, and radiotherapy, except vitamin D insufficiency/deficiency, which was significantly more frequent in pubertal ALL survivors compared to prepubertal ALL survivors (57.5% and 25%, respectively, p=0.011).A high frequency of endocrine complications was observed in the current study. The high frequency of late effects necessitates long-term surveillance of this population to better understand the incidence of late-occurring events and the defining of high-risk features that can facilitate developing intervention strategies for early detection and prevention.Akut lenfoblastik lösemili (ALL) hastalardaki sağ kalım oranlarının artışı, tedavi sonrası ortaya çıkan morbidite ve mortalite problemlerinin takip edilme ihtiyacını beraberinde getirmiştir. Çalışmamızda, en az iki yıldır remisyonda olan ALL’li hastalarda, tedavi sonrası ortaya çıkabilecek endokrin komplikasyonların değerlendirilmesi amaçlandı.ALL tanısı ile tedavi almış ve tedavisi üzerinden en az iki yıl geçmiş ve remisyonda olan 60 hastada endokrin geç komplikasyonlar kesitsel olarak değerlendirildi.Hastaların tanı aldıkları andaki median yaşları 5 yıl (minimum-maksimum: 1,7-13), kemoterapi sonlandırıldığı andaki median yaşları 8 yıl (minimum-maksimum: 4,25-16), çalışma sırasındaki median yaşları ise 11,7 yıl (minimum-maksimum: 7-22) olarak tespit edildi. Hastaların tedavi sonrası median takip süresi ise 4 yıl (minimum-maksimum: 2-10,1) idi. Hastaların %81,6’sında en az bir endokrin komplikasyon geliştiği görüldü. D vitamini eksikliği/yetersizliği (%46,6), obezite/fazla kiloluluk (%33,3) ve dislipidemi (%23,3) en sık gelişen üç komplikasyon olarak tespit edildi. D vitamini eksikliğine sekonder gelişen hiperparatiroidi (%15), insülin direnci (%11,7), hipertansiyon (%8,3), boy kısalığı (%6,7), tiroid fonksiyon bozukluğu (%5), puberte prekoks (%3,3) ve azalmış kemik mineral yoğunluğu (%1,7) gelişen diğer endokrin komplikasyonlardı. Hastalarda gelişen endokrin komplikasyonlar arasında cinsiyet, yaş, radyoterapi bakımından farklılık saptanmaz iken, D vitamini yetersizliği/eksikliği saptanan hasta sayısı pubertal grupta, prepubertal gruba göre anlamlı derecede fazlaydı (%57,5 ve %25, sırasıyla, p=0,011).Çalışmamızda yüksek oranda endokrin komplikasyon saptandı. Bu komplikasyonlar, geç yan etkilerin ortaya çıkmasına neden olabilecek yüksek risk özellikleri ve sıklığı tanımlayabilmek, erken tanı ve önleyici stratejileri geliştirmek açısından hastaların uzun dönem izlenmelerini gerektirmektedir.

Recently, awareness of the cumulative radiation exposure for pediatric oncology patients has been increasing, together with increased survival rates and longer life expectancy. The aim of our study was to quantify the amount of ionising radiation from imaging modalities of pediatric oncology patients.Eighty-eight patients who were diagnosed with childhood cancer and followed up for 5 years between 2004-2014 in our center were included in the study. Patients' medical files were reviewed retrospectively for imaging history in the first 5 years after diagnosis. Total estimated effective doses from radiologic imaging modalities were determined. Also, the basic demographic data, histologic type, stage, and outcomes of disease were collected for all patients.The individual total estimated effective doses ranged from 8.73 to 167 mSv, with a median of 62.92 mSv. Computed tomography was the greatest contributor of total effective doses. The doses ranged 21.45-113.20 mSv (median: 62.92 mSv) in Hodgkin lymphoma, 12.53-167.10 mSv (median: 52 mSv) in non-Hodgkin lymphoma, 4.13-172.98 mSv (median: 52 mSv) in neuroblastoma, 31-149.89 mSv (median: 63.10 mSv) in Wilms' tumor, 11.50-73.72 mSv (median: 36.90 mSv) in germ cell tumor, 26.46-125.86 mSv (median: 80.90 mSv) in other solid tumor and 0.02-13.31 mSv (5.25 mSv) in brain tumor subgroup. Twenty-two children (25%) died with progressive disease during the 5-year follow-up period.Similar to previous studies, the total estimated effective doses in children with cancer have been found various according to diagnosis, stage and clinical course. To clarify the harmfull effects of radiation burden, prospective studies should be conducted in children with cancer.

Aim The aim of this study was to evaluate the clinical presentation, risk factors, complications, treatment and outcomes of cholelithiasis in children. Methods Children with cholelithiasis were reviewed for demographic information, predisposing factors, presenting symptoms, laboratory findings, complications, treatment and outcome, retrospectively. Results A total of 254 children with cholelithiasis (mean age: 8.9 ± 5.2 years) were recruited to the study. Girls (52.8%) were significantly older than boys (P < 0.001). Symptomatic patients (59%) were significantly older than asymptomatic patients (P = 0.002). Abdominal pain was the most frequent symptom. No risk factors were identified in 56.6% of the patients. Ceftriaxone (20%) was the most commonly associated risk factor. At presentation, at least one of the following complications was seen in 14.1% of patients: cholecystitis (10.9%), obstructive jaundice (2.7%), pancreatitis (1.96%) and cholangitis (1.2%). There was no relationship between gallstone size and symptoms, aetiological factors and complications. The cholelithiasis dissolution rate was higher in younger children (P = 0.032), in those with biliary sludge (P < 0.0001) and ceftriaxone-related cholelithiasis (P < 0.001). Haemolytic anaemia (P = 0.001) and older age (P = 0.002) were associated with stable stones. Ursodeoxycholic acid was administered to 94.4% of patients at presentation. Twenty-nine patients underwent cholecystectomy, and seven patients underwent endoscopic retrograde cholangiopancreotography. Patients who were symptomatic at presentation had significantly more frequent symptoms at follow-up (P < 0.001) Conclusions Dissolution rate of cholelithiasis was higher in younger children, biliary sludge formation and ceftriaxone-related cholelithiasis but lower in older children and haemolytic anaemia-related cholelithiasis.

Angiogenesis have implications in leukemia biology. Angiopoietin 1 (Ang 1) is an angiogenic cytokine which is essential in survival and proliferation of endothelial cells. Angiopoietin 2 (Ang 2) promotes dissociation of pericytes and increases vascular permeability and stromal derived factor 1 alpha (SDF 1α) which is a key player in stem cell traffic in the bone marrow (BM), has stimulating effects on angiogenesis as well. Here, we investigated the role of the leukemic BM microenvironment and specifically, the role of SDF 1α-CXCR4 and Ang 1/Ang 2-Tie 2 axes.Here, Ang 1, Ang 2, and SDF 1α levels were measured in the BM plasma and in supernatants of mesenchymal stem/stromal cells (MSCs) of patients with ALL and compared with those of healthy controls.The results showed that at diagnosis, BM plasma levels of Ang 1 and SDF 1α were significantly low and Ang 2 was high when compared to control values. Remission induction was associated with an increase in Ang 1/Ang 2 ratio and SDF levels in BM plasma.The results suggest that BM microenvironment and leukemic cell-stroma interaction influences the secretion of Ang 1, 2 and SDF 1α, thus, may affect both angiogenesis, homing and mobilization of leukemic blasts.

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

PH is a rare condition with high mortality rate after pediatric HSCT. As clinical presentation is non-specific and may mimic other conditions, a high degree of suspicion is required for diagnosis. Here, we present a patient with stage-IV neuroblastoma who developed PAH after autologous HSCT. After exclusion of other causes of PH, we regarded that this condition was secondary to HSCT.

Intrathecal chemotherapy and systemic chemotherapy are used for both prophylaxis and treatment of central nervous system disease in hematologic malignancies. We presented two children with urinary retention due to neurotoxicity with two distinct drugs and reasons. The main presenting complaints were urinary retention and incontinence in both patients. Before the adverse clinical event, the patients had received vincristine intravenously and triple therapy with hydrocortisone, cytosine arabinoside, and methotrexate intrathecally. The clinical event resolved, and vincristine and intrathecal chemotherapy were deleted from the patients subsequent therapy until recovery was achieved.

In this study, we aimed to determine the effect(s) of G-CSF priming on graft and transplantation parameters and compare these findings with those obtained without priming. A total of 64 pediatric patients transplanted from HLA-matched family donors were enrolled in the study. Twenty-nine patients received G-CSF primed marrow (G-BM group) and 35 patients received steady state bone marrow (S-BM group). Number of total nucleated cells (TNC) and CD34(+) cells, CFU-GM colony number, neutrophil and platelet engraftment times, total length of stay in hospital, overall and disease free survival, and occasions of acute and chronic GvHD has been compared between these two groups. Granulocyte colony stimulating factor primed bone marrow (G-BM) yielded higher numbers of CD34(+) cells, TNCs, and CFU-GM colony numbers compared to those obtained in S-BM. The neutrophil engraftment time, platelet engraftment time, length of stay in hospital, overall survival and disease free survival were not different between G-BM and S-BM groups. Also the cumulative incidence of grades II-IV acute and chronic GvHD were similar. It was observed that the use of G-CSF did not increase the risk of acute or chronic GvHD. We concluded that use of G-CSF for stem cell mobilization is an effective and safe method in children.

Bu, combined with TDM-guided dosing, is associated with fewer graft failures/relapses and lower toxicity in pediatric HSCT. We aimed this retrospective study for comparison of weight- and age-based dosing in terms of clinical outcomes such as time to engraftment, early complications, EFS, OS, and toxicity profiles in children receiving iv Bu. Sixty-one children who underwent HSCT from April 2010 to February 2013 by means of a Bu-based conditioning regimen and completed 100 days after transplantation at Ankara Children?s Hematology and Oncology Hospital Bone Marrow Transplantation Unit were enrolled in this study. SOS and neutropenic fever occurred more frequently in the weight-based dosing group. We found a statistically significant correlation between Bu dose and the incidence of SOS (r = 0.26, p = 0.04). Multivariate analysis showed only weight-based dosing of Bu was a significant predictor of SOS (HR = 9.46; p = 0.009). However, no relationship was found between two groups in terms of hemorrhagic cystitis, engraftment syndrome, acute or chronic GvHD, time to engraftment, chimerism, TRM, OS, and EFS rates. Weight-based dosing of Bu may cause higher incidence of SOS and early infectious complications at the places where TDM of Bu cannot be performed.

Background/aim: There are different modalities for management of infantile hemangiomas (IHs). In this report, our aim is to evaluate whether intralesional corticosteroid treatment is associated with systemic side effects and whether this is an effective treatment modality for IH. Materials and methods: Six children treated with intralesional corticosteroids for problematic hemangiomas were included in the study. Clinical characteristics, response to treatment, weight, height, blood pressure, morning serum cortisol, and adrenocorticotropic hormone levels were recorded. Results: Each child received intralesional triamcinolone at a dose of 2 mg/kg for 2?5 injections at monthly intervals. Subjects were followed for 1 year. All patients had adrenal suppression following the second or third triamcinolone injections. Five patients demonstrated partial response and one demonstrated no response. Conclusion: Intralesional steroid injection may effectively induce the resolution of hemangiomas, but all the patients in our group had adrenal suppression after treatment. The use of intralesional steroid therapy is not a superior treatment option for hemangiomas. It also has side effects comparable to systemic steroids.

Cisplatin is one of the effective chemotherapeutic agents widely used for many tumor types in children. The most serious complications of cisplatin overdose are nephrotoxicity and ototoxicity. It may lead to life-threatening complications and even death. We report a 13-year-old female patient with osteosarcoma who received a massive cisplatin overdose mistakenly. She was given cisplatin 120 mg/m2/day for consecutive two days, instead of one day (total 240 mg/m2) due to a prescription error. After 12 hours, her cisplatin level was measured as 8,500 ng/ml (normal: 1,000-5,000 ng/ml). She was treated with repeated plasmapheresis combined with use of two chemoprotectants, (N-acetylcysteine and amifostine). On her follow up, renal functions and audiometric tests remained normal. According to our experience with our case, early urgent combined treatment is very important in the management of cisplatin overdose.

The study was designed to compare colony forming capacity of granulocyte-colony stimulating factor (G-CSF) stimulated bone marrow (G-BM) with standard unstimulated bone marrow (U-BM) of healthy donors of pediatric patients. CFU-Assay results of 26 healthy donors of pediatric patients undergoing allogeneic hematopoietic stem cell transplantation (HSCT) were analyzed retrospectively. 13 of donors received 10 μg/kg per day of G-CSF as a single injection for 3 consecutive days and other 13 of donors had unstimulated BM. Colony forming capacity of hematopoietic stem cells evaluated with Colony Forming Unit-Assay (CFU-Assay) with in semi-solid agar culture medium after 14-18 days of culture period. CFU-Assay results of G-BM and U-BM (expressed as means) were; Burst Forming Unit-Erythroid (BFU-E): 15.20 × 10(4)/kg and 8.38 × 10(4)/kg, Colony Forming Unit-Granulocyte Macrophage (CFU-GM): 10.35 × 10(4)/kg and 5.67 × 10(4)/kg, Colony Forming Unit-Erythroid (CFU-E): 0.59 × 10(4)/kg and 0.33 × 10(4)/kg, CFU-Granulocyte Erythroid Macrophage Megakaryocyte (CFU-GEMM): 0.52 × 10(4)/kg and 0.53 × 10(4)/kg respectively. BFU-E and CFU-GM capacity of G-BM was increased and statistically significantly different than standard U-BM (p ⩽ 0.01). In conclusion, increased colony forming capacity of hematopoietic stem cells of G-BM when compared with standard unstimulated BM could be a major advantage for transplantation.

Background Early life-threatening cardiotoxicity and cardiac death have been reported after hematopoietic stem cell transplantation (HSCT). The purpose of the current study was to evaluate cardiac toxicity of conventional chemotherapy followed by HSCT with cardiac markers: heart-type fatty acid binding protein (H-FABP), glycogen phosphorylase BB (GPBB), high sensitive C reactive protein (hsCRP) cardiac troponin I, (cTnI), creatine kinase MB (CK-MB mass) and myoglobin. Methods A total of 20 children who underwent HSCT for malignant and non-malignant diseases were included in this study. Blood samples were collected from all patients in 0th, 7th and 21st day for evaluating these cardiac biomarkers. The patients’ echocardiography was assessment before and after one-month of HSCT. Results Serum 21st H-FABP level was significantly higher when compared with the 0th day H-FABP level (P < 0.05) . 7th day hsCRP level was significantly higher than 0th and 21st day levels (P < 0.05). Interestingly, 7th day GPBB level was significantly lower than 0th and 21st day levels (P < 0.05). Myoglobin, CK-MB mass and cTnI biomarkers remained within the reference range in all patients. Conclusions This study showed that H-FABP and hsCRP both seem to be promising markers for evaluation of cardiotoxicity in HSCT process and probably superior to GPBB, cTnI, CK-MB mass and myoglobin.

Congenital amegakaryocytic thrombocytopenia (CAMT) generally begins at birth with severe thrombocytopenia and progresses to pancytopenia. It is caused by mutations in the thrombopoietin receptor gene, the myeloproliferative leukemia virus oncogene (c-MPL). The association between CAMT and c-MPL mutation type has been reported in the literature. Patients with CAMT have been categorized according to their clinical symptoms caused by different mutations. Missense mutations of c-MPL have been classified as type II and these patients have delayed onset of bone marrow failure compared to type I patients. Here we present a girl with severe clinical course of CAMT II having a missense mutation in exon 4 of the c-MPL gene who was admitted to our hospital with intracranial hemorrhage during the newborn period.Konjenital amegakaryositik trombositopeni (KAMT) genellikle, doğumda ağır trombositopeni ile başlar ve pansitopeniye ilerler. Hastalığın nedeni trombopoetin reseptör geni olan myeloproliferatif lösemi virüs onkogenindeki (c-MPL) mutasyon olup, literatürde KAMT kliniği ile c-MPL mutasyon tipleri arasında ilişki bildirilmiş, hastalar farklı mutasyonların neden olduğu klinik belirtilere göre sınıflandırılmışlardır. c-MPL’nin yanlış anlamlı mutasyonları tip 2 olarak sınıflanmıştır. Bu hastalar tip 1 hastalar ile karşılaştırıldığında kemik iliği yetmezliğinin daha geç başladığı bildirilmiştir. Burada, yenidoğan döneminde intrakranial kanama ile hastanemize başvuran c-MPL geninde ekzon 4’de yanlış anlamlı mutasyonu olan KAMTII tanılı ağır klinik seyirli bir kız sunulmuştur.

Even the life span has prolonged for the last 40 years, increase in frequently seen complications with increasing age negatively affect the life quality of thalassemia patients. In our study, complications encountered in 67 s-thalassemia patients who were followedup at our hospital between 1 January 2004 and 31 May 2009 were retrospectively analyzed. Fifty-six patients were followed up with diagnosis of thalassemia major and 11 with thalassemia intermedia. Totally, 56.7% of patients were male and 43.3% were female. Ages varied between 2-20 years with the mean age of 10.3±4.8 years. Mean ferritin level was 2212±1370 ng/mL (41-6263 ng/mL) for 4.5 years. Complications were increased with increasing age. Complication rates were significantly higher among thalassemia major patients compared to thalassemia intermedia patients. There was no statistically significant relationship between complications and mean ferritin levels. The most common complications were endocrine complications (38.8%). Cardiac complications developed in 22.4% of the patients; gastroenterological complications in 19.4%; allergic complications in 9%; infectious complications in 1.5%; and thrombosis was detected in 1.5%. The endocrine complications were osteoporosis, growth retardation, developmental delay, short stature, hypothyroidism, delayed puberty, hypogonadism, and diabetes mellitus. The cardiac complications were left ventricular wall hypertrophy, diastolic dysfunction, systolic dysfunction, heart failure, pericardial effusion, dilated cardiomyopathy, left ventricular dilatation, left atrial dilatation, and fatal arrhythmias. Mortality occurred in one (1.5%) out of 67 s-thalassemia patient due to dilated cardiomyopathy and fatal arrhythmia.

The purpose of this study was to evaluate the relationship between the plasma holoTC and serum vitamin B-12 in children and to identify a cutoff cobalamin values according to holoTC. One hundred and fifty-five children were enrolled into the study. All children were evaluated for hemoglobin, vitamin B-12, folate, ferritin and holoTC levels. Children were grouped as with low vitamin B-12 level ( 200 pg/mL, group II). Serum vitamin B-12, and holoTC levels were performed in each patient in the study. In 101 patients with low vitamin B-12 (group I) the mean holoTC was 21.74 +/- 1.14 pmol/L. In 54 children with normal vitamin B-12 (group II) mean holoTC was 44.0 +/- 2.7 pmol/L (p < 0.01). A ROC curve analysis was performed to delineate the optimum cut-off point for vitamin B-12 level and it was found to be 165 pg/mL with a sensitivity of 70% and specificity of 74%; the area under curve was 0.783 (p < 0.01). Our study displayed a positive correlation between vitamin B-12 and holoTC, and defined an optimum cutoff value for vitamin B-12 as 165 pg/mL. Further studies using the markers both MMA, tHcy and holoTC to confirm the findings are needed.

BACKGROUND The purpose of this study is to determine early myocardial dysfunction in β-thalassemia major (BTM) patients. Where the myocardial dysfunction cannot be detected by conventional echocardiography, it could be detected by tissue Doppler imaging (TDI) or speckle tracking echocardiography (STE). METHODS In this study, we analyzed 60 individuals, 30 of whom were BTM patients and the other 30 of whom were the control group. T2* magnetic resonance imaging (MRI) was used to measure cardiac iron deposition. The myocardial functions were evaluated by conventional echocardiography, TDI and STE. RESULTS When basal lateral left ventricular and basal septal wall TDI values were compared between the patient group and control group, only isovolumic contraction time values were significantly longer in the patients. The global circumferential strain was significantly lower in the patients. When evaluated as segmental, longitudinal strain values of basal inferoseptum and circumferential strain values of anteroseptum, anterior, and inferolateral segments were significantly lower in the patients. In the patients, global longitudinal and circumferential strains in the group who had pathological T2* values were significantly lower than the group who did not. In addition, circumferential strain values in anteroseptum, anterolateral, inferior, and inferoseptum segments were significantly lower in the patients with T2* values

Gastrointestinal tract is one of the major systems affected by graft-versus-host disease (GVHD). Injury to the gut during conditioning therapy before stem-cell transplantation (SCT) plays a pivotal role in the initiation of inflammatory stimuli. We reviewed medical records of the patients who underwent SCT between April 2010 and June 2013 in our center. A stepwise upgrade diet was given to the children with acute GI-GVHD (Gastrointestinal GVHD) including parenteral and enteral nutrition. A total of 105 patients underwent SCT and seven patients developed grade III-IV acute GI-GVHD. Total parenteral nutrition (TPN) was initiated to all patients after the diagnosis of GI-GVHD and minimal enteral nutrition (1-2 ml/kg/day standard pediatric enteral formula/special meat soup) was given to the patients. GI-GVHD improved in all patients with no change in body weight, and recovery to a normal diet took 10-30 days. Stepwise diet management of oral nutrition contributed to rapid improvement of grades III-IV acute GI-GVHD.

The aim of this study was to evaluate nutritional status in children who underwent hematopoietic stem cell transplant compared with a healthy control group. A secondary aim was to utilize mid-upper arm circumference as a measure of nutritional status in these groups of children.Our study group included 40 children (18 girls, 22 boys) with mean age of 9.2 ± 4.6 years (range, 2-17 y) who underwent hematopoietic stem cell transplant. Our control group consisted of 20 healthy children (9 girls, 11 boys). The children were evaluated at admission to the hospital and followed regularly 3, 6, 9, and 12 months after discharge from the hospital.In the study group, 27 of 40 patients (67.5%) received nutritional support during hematopoietic stem cell transplant, with 15 patients (56%) receiving enteral nutrition, 6 (22%) receiving total parenteral nutrition, and 6 (22%) receiving enteral and total parenteral nutrition. Chronic malnutrition rate in the study group was 47.5% on admission to the hospital, with the control group having a rate of 20%. One year after transplant, the rate decreased to 20% in the study group and 5% in the control group. The mid-upper arm circumference was lower in children in the study group versus the control group at the beginning of the study (P.05). However, there were no significant differences in mid-upper arm circumference measurements between groups at follow-up examinations (P.05). During follow-up, all anthropometric measurements increased significantly in both groups.Monitoring nutritional status and initiating appropriate nutritional support improved the success of hematopoietic stem cell transplant and provided a more comfortable process during the transplant period. Furthermore, mid-upper arm circumference is a more sensitive, useful, and safer parameter that can be used to measure nutritional status of children who undergo hematopoietic stem cell transplant.

The aim of this study was to investigate the effects of donor characteristics on CD34+ cell yield in BM harvest. Between April 2010 and November 2013, consecutive donors who underwent BM harvesting in our BM transplantation unit were retrospectively investigated. Donors were classified into two groups: those who donated BM without mobilization (steady-state BM donors) and those who received G-CSF for stem cell mobilization (G-CSF-primed BM donors). Donor characteristics (age, gender, race, body weight, BMI, and laboratory factors including donor's leukocyte, platelet, and monocyte) and their relationship with total nuclear cell and CD34+ cell numbers has been evaluated. A total of 64 healthy related donors (29 males/35 females, median age 11.2 years; 49 [76.6%] younger than 18 and 36 [56.3%] younger than 12 years) were included in the study. The median CD34+ cell yield in the harvest was 0.12×106/L (0.02-0.21) in SS-BM donors and 0.18×106/L (0.09-0.67) in GP-BM donors (P=.03). Median of CD34+ cell count given to recipients was 2.6×106/recipient body weight (1.3-19.3) in SS-BM yields and 3.8×106/recipient body weight (1.1-10.2) in GP-BM yields, respectively. Multiple regression analysis showed that donor height and pre-G-CSF platelet were the most important parameters to obtain a sufficient BM harvest. Our data suggest that the shorter donors and the donors with higher thrombocyte counts may offer more hematopoietic stem cell. The height and thrombocyte count of the donors should be taken into consideration before planning the targeted CD34+ cell count especially for pediatric donors.

Background:Endocrine organs are highly susceptible to effects of high-dose chemotherapy. The objective of the study was to evaluate endocrine and metabolic complications after hematopoietic stem cell transplantation (HSCT) in children.Methods:The patients who underwent HSCT in our center from April 2010 to October 2014 with at least 1 year follow-up were analyzed retrospectively.Results:One-hundred children (M/F:59/41; mean age 8.9±4.8 years, mean follow-up time 3.4±1.2 years) were included in the study. Female hypogonadism was the most common endocrine dysfunction (35.7%), followed by growth impairment (29.4%), malnutrition (27.4%), dyslipidemia (26%), low bone mineral density (BMD) (25%), hypothyroidism (13%) and insulin resistance (12%). Patients who underwent HSCT >10 years of age were significantly at risk for hypogonadism, metabolic syndrome, growth impairment and malnutrition (pConclusions:Endocrine or metabolic dysfunctions are more prevalent in children who are older than 10 years of age at HSCT. Children who underwent HSCT should be followed-up by a multidisciplinary team during puberty and adolescence.

Primer kutanöz lenfomalar çocukluk çağında çok nadir olup histolojik alt tip, klinik seyir ve prognoz bakımından farklılık gösterirler. Burada farklı histopatolojik özelliklere sahip iki primer kutanöz lenfoma olgusu sunulmaktadır. Her iki olgunun ortak özelliği sistemik antibiyotik ve pansuman tedavilerine yanıt vermeyen cilt apsesi ile başvurmuş olmalarıydı. Biyopsi ve evreleme çalışmalarından sonra bir olgu primer kutanöz periferik T hücreli lenfoma, tanımlanamayan, diğer olgu ise primer kutanöz CD30 pozitif ALK negatif anaplastik büyük hücreli lenfoma tanısı aldı., Primary cutaneous lymphomas in children are rare and constitute a heterogeneous group in regard to histological subtype, clinical course and prognosis. Here, we present the clinical characteristics and outcomes of two children with primary cutaneous lymphoma with different histopathologic subtypes. They had been diagnosed as a skin abscess initially but had not responded to systemic antibiotherapy and dressing. After a biopsy and metastatic work up, one patient was diagnosed as primary cutaneous peripheral T-cell lymphoma, unspecified, and the other as primary cutaneous CD30 positive ALK negative anaplastic large cell lymphoma

The aim of this study was to evaluate the clinical presentation, risk factors, complications, treatment and outcomes of cholelithiasis in children.Children with cholelithiasis were reviewed for demographic information, predisposing factors, presenting symptoms, laboratory findings, complications, treatment and outcome, retrospectively.A total of 254 children with cholelithiasis (mean age: 8.9 ± 5.2 years) were recruited to the study. Girls (52.8%) were significantly older than boys (P0.001). Symptomatic patients (59%) were significantly older than asymptomatic patients (P = 0.002). Abdominal pain was the most frequent symptom. No risk factors were identified in 56.6% of the patients. Ceftriaxone (20%) was the most commonly associated risk factor. At presentation, at least one of the following complications was seen in 14.1% of patients: cholecystitis (10.9%), obstructive jaundice (2.7%), pancreatitis (1.96%) and cholangitis (1.2%). There was no relationship between gallstone size and symptoms, aetiological factors and complications. The cholelithiasis dissolution rate was higher in younger children (P = 0.032), in those with biliary sludge (P0.0001) and ceftriaxone-related cholelithiasis (P0.001). Haemolytic anaemia (P = 0.001) and older age (P = 0.002) were associated with stable stones. Ursodeoxycholic acid was administered to 94.4% of patients at presentation. Twenty-nine patients underwent cholecystectomy, and seven patients underwent endoscopic retrograde cholangiopancreotography. Patients who were symptomatic at presentation had significantly more frequent symptoms at follow-up (P0.001) CONCLUSIONS: Dissolution rate of cholelithiasis was higher in younger children, biliary sludge formation and ceftriaxone-related cholelithiasis but lower in older children and haemolytic anaemia-related cholelithiasis.

Background and Objectives Candida-associated bloodstream infections are frequent and potentially life-threatening conditions in hematology patients. The aim of this study is to evaluate the characteristics, risk factors, and outcome of Candida-associated bloodstream infections in children with hematological diseases. Methods The medical records of the patients with hematological diseases and hematopoietic stem cell transplantation (HSCT) recipients who were diagnosed as Candida-associated bloodstream infection between February 2010 and February 2014 were reviewed retrospectively. Results Thirty episodes of candidemia involving 26 patients (38% female, and 62% male) with a median age of 7-year (range; 1 to 17) were noted. The incidence of candidemia in our study was 5.2 per 1000 hospital admissions. Infections with non-albicans Candida spp. occurred more frequently (63%) and C. krusei was the predominant microorganism among non-albicans Candida spp. (37%). Candida albicans was isolated from 11 of the 30 episodes (37%). Twenty-six of the episodes (88%) patients had a central venous catheter (CVC) prior to candidemia, and they were removed in 16 (62%). Thirty-day mortality rate was 20%. Isolated Candida spp, underlying disease and its status, presence of mucositis, neutropenia, using of broad spectrum antibiotics, corticosteroids or total parenteral nutrition were not identified as predictors of outcome. Multivariate analysis revealed that CVCs kept in place was the only significant factor associated with mortality (OR, 0.07; 95% CI, 0.006–0.716). Conclusions Candida-associated bloodstream infections were common in children with hematological diseases and HSCT recipients, particularly in patients with CVCs. In addition to appropriate antifungal therapy, CVC removal improves the outcome of candidemia in children with hematological disease.

Thirty-nine children with Fanconi aplastic anemia (FAA) have been followed up in our center between January 2008 and November 2010. Eight of these children (20%) with a transfusional iron overload had been undergoing deferasirox treatment during the study period. In the English literature, transfusional iron overload and the use of an iron chelator in children with FAA has not yet been evaluated. Here, we have presented the effectivity and tolerability of deferasirox in children with FAA and a transfusional iron overload. Before the deferasirox treatment, the mean serum ferritin level was 3377 ± 2200 ng/mL. After a mean 13.6-month treatment duration, the mean ferritin level decreased to 2274 ± 1300 ng/mL (P

Asparaginase, an effective drug in the treatment of childhood acute lymphoblastic leukemia (ALL), has become an important component of most childhood ALL regimens during the remission induction or intensification phases of treatment. The incidence range of asparaginase-associated lipid abnormalities that are seen in children is 67-72%. Lipemia causes erroneous results, which uses photometric methods to analyze blood samples. We describe a case of l-asparaginase-associated severe hyperlipidemia with complete blood count abnormalities. Complete blood count analysis was performed with Beckman COULTER(®) GEN·S™ system, which uses the Coulter Volume, Conductivity, Scatter technology to probe hydrodynamically focused cells. Although an expected significant inaccuracy in hemoglobin determination occurred starting from a lipid value of 3450 mg/dl, we observed that triglyceride level was 1466 mg/dl. Complete blood count analysis revealed that exceptionally high hemoglobin, mean corpuscular hemoglobin, and mean corpuscular hemoglobin concentration levels vs. discordant with red blood cell count, mean corpuscular volume, and hematocrit levels. Total leukocyte count altered spontaneously in a wide range, and was checked with blood smear. Platelet count was in expected range (Table 1). Thus, we thought it was a laboratory error, and the patient's follow-up especially for red cell parameters was made by red blood cell and hematocrit values.

Pyomyositis is a primary infection of the skeletal muscles, usually present as single or multiple abscess formation. Early diagnosis is frequently difficult in nontropical countries because of the ...

All-trans-retinoic acid (ATRA), which is used in acute promyelocytic leukemia, is usually well tolerated, but some side effects can be observed. Retinoic acid syndrome is the most severe side effect. Triazole derivatives such as fluconazole inhibit the NADPH-dependent cytochrome P-450-mediated catabolism of ATRA and are increased plasma levels of ATRA. Here, the authors report a case of APL who developed acute renal failure during ATRA and concurrent use of fluconazole.

In childhood, pulmonary thromboembolism (PTE) is an uncommonbut potentially life-threatening disease. The greater numbers of venous thromboembolism (VTE) are complications of underlying risk factors such as malignancies, chemotherapy (L-asparaginase), and central venous catheter. We report a patient with acute lymphoblastic leukemia and PTE, who presented with near-syncope, and was successfully treated with low molecular weight heparin and calcium channel blockers.