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Background: Total body irradiation (TBI) is the cornerstone of conditioning regimens in pediatric hematopoietic stem cell transplantation for acute lymphoblastic leukemia. As the late effects and survival comparison between TBI and chemotherapy were well analyzed before, in this study, we aim to focus on the first 100 days and early complications of transplantation. Methods: This retrospective study involves 72 pediatric patients (0 to 18 y) underwent first hematopoietic stem cell transplantation for acute lymphoblastic leukemia between October 2015 and May 2019. Patients are divided into 2 groups regarding conditioning regimens. Conditionings includes either TBI 1200 cGy/6 fractions/3 days and etoposide phosphate or busulfan, fludarabine, and thiotepa. Busulfan was administered IV and according to body weight. Results: The incidences of acute graft versus host disease grade 2 to 4, veno-occlusive disease, capillary leakage syndrome, thrombotic microangiopathy, blood stream infection, hemorrhagic cystitis and posterior reversible encephalopathy syndrome before day 100 were similar for both conditioning regimens; however, patients received TBI-based conditioning had significantly longer neutrophil engraftment time (17.5 vs. 13 d, P=0.001) and tended to have more engraftment syndrome (ES) (45.5% for TBI vs. 24.0% for chemotherapy, P=0.069). Multivariate analysis showed that TBI-based conditioning was associated with a longer neutrophil engraftment time (hazard ratio [HR]=1.20, P=0.006), more cytomegalovirus (CMV) reactivation (HR=3.65, P=0.038) and more ES (HR=3.18, P=0.078). Conclusions: Our findings support chemotherapy-based regimens with early neutrophil engraftment, less ES and CMV reactivation compared with TBI. Although there is no impact on survival rates, increased incidence of ES and CMV reactivation should be considered in TBI-based regimens.

Large KH defects involving both upper and lower laminae and facets can expose the roots to greater extent than small KH defects at C3-4, C5-6, and C6-7 levels. Large KH defects may allow better exposure of nerve roots axillae than small KH defects.

Objectives: The main purpose of this study was to determine a comparison between the chondroprotective efficacy of intra-articular administrations of glucosamine sulphate (GS) and hyaluronic acid (HA) in the experimental knee osteoarthritis model in rats. Materials and Methods: Forty-five rats were assigned to the following three groups: GS group (Group 1), HA group (Group 2), control group (Group 3). The knee joints of the rats were seen macroscopically and the anterior cruciate ligaments were resected. Two weeks after surgery, an intra-articular injection was applied to each group for three weeks with 1 week interval. In the osteoarthritic rat knee joints, the histopathological effects of tissue regeneration of GS and HA applications were compared with the control group. Results: The total Mankin Scale was 2.64±2.56, 3.58±3.9, 8.12±2.80 for the GS group, HA group and control group respectively. According to these results, the GS and HA were superior to the control group. The values of the total Mankin Scale, cartilage structure, cellular abnormality, matrix staining and tidemark integrity of the GS group were lower than the values of the HA group but this difference was not statistically significant. Conclusion: According to the results of this study, intra-articular administration of GS for the management of osteoarthritis may be beneficial for patients with knee osteoarthritis.

Insulin receptor (INSR) mutations lead to heterogeneous disorders that may be as severe as Donohue syndrome or as mild as "type A insulin resistance syndrome". Patients with severe disorders usually harbor homozygous or compound heterozygous mutations. In contrast, type A insulin resistance syndrome has been associated with heterozygous mutations; homozygous mutations are rarely responsible for this condition. We report a novel, homozygous mutation, p.Leu260Arg in exon 3, of the INSR gene in a female adolescent patient with type A insulin resistance syndrome together with clinical details of her medical follow-up. Different mutations in the INSR gene cause different phenotype and vary depending on the inheritance pattern. This report adds to the literature, increases understanding of the disease mechanism and aids in genetic counseling.