7-12.1, P < .01), respectively. These results indicate that the HCT-CI score predicts NRM and OS in pediatric patients undergoing HCT and is a useful tool to assess risk, guide MK-2206 inhibitor counseling in the pre-transplantation setting, and devise innovative therapies
for the highest risk groups. (Blood. 2011; 117(9): 2728-2734)”
“Background: Sturge-Weber syndrome is a congenital neurocutaneous disorder characterized by facial port-wine stain, leptomeningeal angioma, and neurological disorders. Sturge-Weber syndrome can coexist with other disorders in a few patients; however, muscular abnormalities have not been reported in patients with Sturge-Weber syndrome.\n\nCase presentation: A Chinese girl presented with extensive port-wine stains, congenital bilateral glaucoma, and leptomeningeal angiomatosis. The neurocutaneous symptoms were consistent with the diagnostic criteria of BAY 80-6946 PI3K/Akt/mTOR inhibitor Sturge-Weber syndrome. Meanwhile, episodes of rhabdomyolysis were supported by the recurrent symptoms as follows: exercise intolerance, hyperCKmia,
elevated serum myoglobin, and renal failure. Myopathological features and high level of blood long-chain acyl-carnitine indicated that episodes of rhabdomyolysis might be caused by lipid metabolic myopathy. Causative mutations were not ASP2215 found in the CPT2, ACADVL, and GNAQ gene.\n\nConclusions: We report the first case that Sturge-Weber syndrome coexists with episodes of rhabdomyolysis associated with lipid metabolic myopathy.”
“Purpose: To evaluate
the gonial angle (GA) and associated factors that can contribute to stability after bilateral sagittal split ramus osteotomy setback and Le Fort I advancement osteotomy for the treatment of patients with mandibular excess.\n\nMaterials and Methods: This retrospective study included 42 randomly selected, adult patients. Lateral cephalometric radiographs were obtained before and 1 week and 1 year after surgery. Patients in group 1 (n = 18) had a GA smaller than 125 degrees and those in group 2 (n = 24) had a GA larger than 125 degrees. Data were analyzed by analysis of variance and Pearson correlations. Multivariate linear regression analysis was used to identify factors that influenced postsurgical stability.\n\nResults: Mean surgical changes were similar in the 2 groups. The mandible was set back an average of 5.4 mm in group 1 and 6.4 mm in group 2, whereas the maxilla was advanced 2.5 mm in group 1 and 1.7 mm in group 2. Statistically significant postoperative changes were noted for group 1 only.