Clinical features and risk factors of hepatic sinusoidal obstruction syndrome in children after hematopoietic stem cell transplantation: A single-center experience.

Hepatic sinusoidal obstruction syndrome (SOS) is an illness with serious life effects that develops after hematopoietic stem cell transplantation (HSCT). We investigated the risk factors and clinical features of hepatic SOS in children following HSCT in 210 children who underwent allogeneic or autologous HSCT between 2009 and 2021 were analyzed in the context of SOS. The syndrome developed in 22 (10.4%) patients:frequently in neuroblastoma [24% (5/21)], hemophagocytic lymphohistiocytosis [57% (4/7)], and thalassemia major [22% (7/31)]. The median time from HSCT to diagnosis was 16 (6-38) days. Severe disease occurred in 8 (36%) patients, and mild/moderate in 14 (64%) and 4 patients died (18%). In univariate analyses, patient's age ≤ 2 years [odds ratio (OR)= 3.043, P = 0.028], pretransplant AST and ALT levels > 100 U/L (OR=3.576, P = 0.045), and chemotherapy/radiotherapy to abdomen before transplantation (OR = 3.162, P = 0.044) were determined as risk factors. In multivariate analysis, pre-transplant AST and ALT levels > 100 U/L (OR = 16.04, P = 0.010) and ferritin levels over 1000 mg/dl (OR=5.15, P = 0.047) were significant. The only independent risk factor on mortality was the age ≤ 2 years (P = 0.001). Although our study confirmed several risk factors for SOS, we failed to achieve some well-known risk factors. Precautions should be taken considering the factors affecting liver function before transplantation and the risk of SOS in infants receiving chemotherapy and radiotherapy before transplantation, such as neuroblastoma in which comparable results in respect to the chemotherapy only. The risk factors should be fully elucidated in multicenter studies to improve preventive and therapeutic strategies.

Treatment Outcomes of Childhood Medulloblastoma with the SIOP/UKCCSG PNET-3 Protocol.

OBJECTIVES: To retrospectively compare the overall and event-free survival rates of patients with standard and high risk medulloblastoma who received postoperative radiotherapy (RT) followed by maintenance chemotherapy. METHODS: The study included 48 patients with medulloblastoma who were treated and followed-up between 2005 and 2021. Patients were classified according to the Chang classification because no molecular analysis was done. Immediately after surgery all patients received postoperative RT followed by eight cycles of chemotherapy (SIOP/UKCCSG PNET-3 protocol); if thrombocytopenia developed, carboplatin was replaced by cisplatin to avoid treatment delay. The clinical characteristics, risk categories and treatment outcomes of all patients were analyzed. RESULTS: The mean age of the 48 patients (26 males, 22 females) at diagnosis was 7.27±4.21 y. The median start time of RT after surgery was 37 (range 19-80) d. The median follow-up was 56 (3-216) mo. The 5-year event-free survival was 61.2±10% in the high-risk group and 82.5±11.5% in the standard-risk group. The 5-year overall survival was 73.2±7.1%; it was 61.2±10% and 92.9±6.9% for high- and standard-risk patients, respectively (p = 0.026). CONCLUSIONS: The outcomes of patients who were started on the modified SIOP/UKCCSG PNET-3 chemotherapy protocol, in which RT was begun as soon as possible after surgery, were comparable to those of current treatment protocols. Although a definitive conclusion is difficult, given the limited number of patients in the present study, authors suggest that their treatment protocol is a viable option for centers with limited facilities (such as an inability to perform molecular analysis).

Frequency of obesity and metabolic syndrome in childhood leukemia and lymphoma survivors.

OBJECTIVES: In this study, it was aimed to determine the prevalence and clinical features of obesity and metabolic syndrome, which are long-term effects of survivors after treatment in children with leukemia and lymphoma. PATIENTS AND METHODS: Patients with leukemia and lymphoma, who were diagnosed between 2000 and 2012 (at least 2 two years after remission) were included. Data obtained through reviewing the family history, demographic characteristics, anthropometric measurements, and laboratory parameters (blood glucose, lipid, and insulin levels) were analyzed and compared at the time of diagnosis, after the treatment and at time of the study. RESULTS: Eighty nine patients (45 boys, 44 girls) were included (mean age: 14.7 ± 4.3 years): 77.5% had acute lymphoblastic leukemia, 11.2% had acute myeloid leukemia, and 11.2% had lymphoma. Overall, 46% patients had received radiotherapy, 7% had undergone surgery, and 2.2% had received stem cell transplantation in addition to chemotherapy. The mean duration of treatment was 2.4 years, and the time elapsed after treatment was 4.9 years. While only one had obesity at the diagnosis, a significant increase in obesity (20%), hypertension (15.7%), hyperglycemia (15%), insulin resistance (35%) were observed at the time of study, and family history of hypertension, dyslipidemia, and cardiovascular diseases were significantly higher in this subgroup. CONCLUSION: The prevalence of metabolic syndorme is higher in children with leukemia and lymphoma after treatment, and begins to increase with the initiation of treatment and continues to increase over time. These children should be followed-up for late-effects including metabolic syndrome through life-long period.

3 Tesla MRI in diagnosis and follow up of children with pneumonia.

PURPOSE: To investigate the utilization of 3-Tesla (3 T) magnetic resonance imaging (MRI) in detection of pulmonary abnormalities in children with pneumonia. MATERIALS AND METHODS: Forty-seven children with pneumonia prospectively underwent 3 T thoracic MRI and posteroanterior (PA) chest radiography (CR). Of these, 15 patients also underwent contrast-enhanced thorax computed tomography (CT) or high-resolution CT (HRCT). The MRI protocol included axial and coronal T2-weighted spectral presaturation with inversion recovery (SPIR) Multivane-XD and axial echo-planar diffusion-weighted imaging (EPI DWI) with respiratory gating. Kappa statistics, Cochran Q, and McNemar tests were used to investigate the results. RESULTS: Agreement between CR and MRI was substantial in detecting consolidation/infiltration (k = 0.64), peribronchial thickening (k = 0.64), and bronchiectasis (k = 1); moderate in detecting cavity (k = 0.54) and pleural effusion (k = 0.44); and fair in detecting empyema (0.32) and bilateral involvement of lungs (k = 0.23). MRI was superior to CR in detecting bilateral involvement (p < 0.001), lymph node (p < 0.001), pleural effusion (p < 0.001), and empyema (p = 0.003). MRI detected all the consolidation/infiltration also detected on CT imaging. A kappa test showed moderate agreement between MRI and CT in detecting pleural effusion and ground-glass opacity (GGO), and substantial or almost perfect agreement for all other pathologies. No statistically significant difference was observed between MRI and CT for detecting pneumonia-associated pathologies by the McNemar test. CONCLUSION: Thoracic 3 T MRI is an accurate and effective technique for evaluating children with pneumonia. MRI detected more pathologies than CR and had similar results to those of thorax CT.