Regulation of hematopoiesis II: the role of polyamine inhibition on helper or suppressor influences of the thymus

We have previously suggested in murine model systems, that two cell subpopulations with differing proliferative capacity, from the thymus, modify the growth of erythroid progenitor cells in vitro. In order to further characterize these populations, we have specifically inhibited polyamine biosynthesis; this pathway is essential for the process of cell replication. Thus, alpha-difluoromethyl ornithine (DFMO) was used to block the conversion of ornithine to putrescine, the first and rate- limiting step in polyamine biosynthesis. We observed a threefold increase in hematopoietic progenitors (CFU-S and CFU-E) from bone marrow in animals treated with DFMO. We further examined the effect of DFMO on accessory "helper" and "suppressor" cells from the thymus and observed an increase in helper activity with an elimination of suppressor activity. All of these effects of DFMO were specific for inhibition of polyamine biosynthesis, since simultaneous addition of the depleted biosynthetic product, putrescine, restored suppressor activity. We conclude that polyamine biosynthesis is required acutely for accessory cell regulation of hematopoiesis.     

THE 6‐minute walk test and other endpoints in Duchenne muscular dystrophy: Longitudinal natural history observations over 48 weeks from a multicenter study

Introduction: Duchenne muscular dystrophy (DMD) subjects ≥5 years with nonsense mutations were followed for 48 weeks in a multicenter, randomized, double‐blind, placebo‐controlled trial of ataluren. Placebo arm data (N = 57) provided insight into the natural history of the 6‐minute walk test (6MWT) and other endpoints. Methods: Evaluations performed every 6 weeks included the 6‐minute walk distance (6MWD), timed function tests (TFTs), and quantitative strength using hand‐held myometry. Results: Baseline age (≥7 years), 6MWD, and selected TFT performance are strong predictors of decline in ambulation (Δ6MWD) and time to 10% worsening in 6MWD. A baseline 6MWD of <350 meters was associated with greater functional decline, and loss of ambulation was only seen in those with baseline 6MWD <325 meters. Only 1 of 42 (2.3%) subjects able to stand from supine lost ambulation. Conclusion: Findings confirm the clinical meaningfulness of the 6MWD as the most accepted primary clinical endpoint in ambulatory DMD trials. Muscle Nerve 48 : 343–356, 2013     

脑内窥镜简易三维立体定向治疗颅内血肿68例

1 临床资料唐都医院神经外科自1998年以来,采用VNK-3型纤维脑内窥镜,以CT或磁共振(MR)简易定位碎吸治疗颅内血肿68(男42,女26)例,年龄6～80岁.自发性颅内血肿33例,外伤性颅内血肿35例,发病距入院时间30 min～6 mo.入院时意识清楚26例,意识不清42例,肢体功能障碍64例.CT或MR检查单侧脑内血肿34例,硬膜外血肿15例,硬膜下血肿 12例,硬膜外、硬膜下多发血肿7例,出血量30～150 mL.入院15 min ～24 h内手术,21例成人清醒者局麻手术,11例小儿基础加局麻,36例昏迷者行全麻插管.     

微血管减压术治疗三叉神经痛和面肌痉挛

1对象和方法1.1对象200003/200405我们采用微血管减压术(Micro vasculardecompression,MVD)在唐都医院治疗患者24(男17,女7)例;年龄36～58(43±3)岁;病程2.5～12(7.5±2.3)a.其中三叉神经痛18例,左侧疼痛11例,右侧疼痛7例;疼痛分布于第Ⅰ支2例,第Ⅱ支4例,第Ⅲ支3例,第Ⅰ、Ⅱ支2例,第Ⅱ、Ⅲ支6例,第Ⅰ、Ⅱ、Ⅲ支1例.面肌痉挛发生在右侧5例,左侧1例.所有病例均经苯妥英钠、卡马西平等药物治疗,或者经射频、封闭等手术治疗,疗效不佳,仍有剧烈疼痛,面肌痉挛复发.所有患者术前经常规MRI检查可见神经血管异常征象,表现为神经的变形、移位及压迹…