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??Objective To explore the value of baseline erythrocytic hydrogen sulfide production level in predicting the long-term survival of postural tachycardia syndrome??POTS?? children treated with midodrine hydrochloride. Methods Twenty-eight children??male 12??female 16?? with POTS were included in the study??and all of them were diagnosed with POTS in Department of Pediatrics??Peking University First Hospital from 2009 to 2012. Their mean age was??11.5±2.5??years old. They were treated with midodrine hydrochloride. Twenty-eight children with POTS were divided into two groups according to the baseline erythrocytic hydrogen sulfide production level. Group??consisted of 10 POTS children whose erythrocytic hydrogen sulfide production level was less than or equal to 27.1 nmol/??min·108 erythrocytes????and group ?? consisted of 18 POTS children whose erythrocytic hydrogen sulfide production level was higher than 27.1 nmol/??min·108 erythrocytes??. The mean follow-up time was??5.3±1.8??years. The orthostatic intolerance symptom score and the symptom-free survival were compared between the two groups by Kaplan-Meier curve. Results After 5.5 years of follow-up??the symptom score of children in group ?? was markedly higher than that of group ??χ2=3.458??P??0.05??. The symptom-free survival of group ?? was lower than that of group ?? ??χ2=10.848??P??0.05??. Conclusion The baseline erythrocytic hydrogen sulfide production level is an useful indicator in predicting the long-term survival of POTS children treated with midodrine hydrochloride.     

心房扑动和颤动患者频消融术后发生心脏损伤后综合征一例

患者女性,63岁.在阵发性心房颤动和扑动射频消融术后发生心包压塞,心包引流出血停止2周后,再次发生心包及双侧胸腔积液,糖皮质激素治疗反应良好,考虑为心脏损伤后综合征.     

儿童心力衰竭药物治疗进展与展望

儿童心力衰竭（心衰）是儿科领域急危重症，多数心衰患儿经过传统抗心衰药物治疗后病情仍进展，亟待拓展新的治疗靶点药物。近年来心衰药物治疗领域取得了较大的进展，针对病理生理学发病机制为靶点的新型抗心衰治疗药物在临床上取得了较好的疗效，提供了新的治疗选择。该文通过综述儿童心衰的最新药物治疗进展，并对药物治疗前景进行展望，以期为儿童心衰的药物治疗提供参考。     

危重患儿的输血策略

输血是临床治疗中的一种重要手段，但在实际工作中，尚缺乏指导危重患儿血液输注的相关策略。该文从输血指征、输血风险、输血管理等方面探讨适合危重患儿的输血策略，以提高儿科医师对合理输血的认识和应用安全性。     

国产3D腹腔镜用于住院医师进阶整合二段式腹腔镜模拟培训效果分析

目的 探讨国产3D腹腔镜用于住院医师腹腔镜外科基本技能培训的可行性及效果。方法 选择2019年12月至2021年12月在上海交通大学医学院附属瑞金医院接受进阶整合二段式腹腔镜模拟培训一阶段课程的35名外科住院医师作为研究对象。学员分别操作传统2D腹腔镜与国产3D腹腔镜，采用问卷调查法收集学员对于3D腹腔镜的主观感受。培训后评估操作考核结果。结果 培训过程中部分学员操作3D腹腔镜会产生一定的眼疲劳及眩晕等视觉不适，但对于3D腹腔镜的总体主观感受无明显不适。3D腹腔镜在纵深感（77.1%）、清晰度（62.9%）、空间定位感（80.0%）等方面明显优于2D腹腔镜。学员分别操作3D腹腔镜和2D腹腔镜完成轨道过弯、穿隧道及高低柱3个模块的考核，在用时［（286.6±215.2）s vs. （302.3±249.2）s，（280.1±157.1）s vs. （306.3±248.4）s，（317.2±130.2）s vs. （342.0±283.7）s］、不规范操作次数［（2.3±1.8）次 vs. （2.7±3.3）次，（2.6±2.0）次 vs. （3.1±4.3）次，（7.0±3.8）次 vs. （6.0±9.5）次］及评分［（3.6±0.9）分 vs. （3.5±0.9）分，（3.7±0.9）分 vs. （3.6±0.9）分，（3.6±0.9）分 vs. （3.7±1.0）分］方面，3D腹腔镜组与2D腹腔镜组的客观考核结果差异无统计学意义（P>0.05）。结论 国产3D腹腔镜在腹腔镜外科基本技能培训中有效、可行，学员主观接受程度良好，可以作为在临床工作使用3D腹腔镜前的辅助培训，推进3D腹腔镜的临床应用。     

脊髓性肌萎缩症患者的营养管理

脊髓性肌萎缩（spinal muscular atrophy，SMA）是一种严重的常染色体隐性遗传病，积极的营养治疗可改善SMA患者的营养代谢和体成分，提高药物疗效，保护骨骼及器官功能，提高生存质量及存活率。文章结合国内外经验，分析SMA患者的常见营养相关合并症，如体重和体成分的异常、吞咽障碍、胃排空延迟、胃食管反流和便秘、骨密度下降、维生素和矿物质缺乏、脂肪代谢紊乱、糖代谢紊乱，探讨营养诊断和干预对策，希望SMA患者的营养管理能得到更多的医生、患者及家属的关注。     

Formation of pyrophosphate-like adducts from nerve agents sarin, soman and cyclosarin in phosphate buffer: implications for analytical and toxicological investigations

Phosphate buffer is frequently used in biological, biochemical and biomedical applications especially when pH is to be controlled around the physiological value of 7.4. One of the prerequisites of a buffer compound among good buffering capacity and pH stability over time is its non-reactivity with other constituents of the solution. This is especially important for quantitative analytical or toxicological assays. Previous work has identified a number of amino alcohol buffers like TRIS to react with G-type nerve agents sarin, soman and cyclosarin to form stable phosphonic diesters. In case of phosphate buffer we were able to confirm not only the rapid hydrolysis of these agents to the respective alkyl methylphosphonates but also the formation of substantial amounts of pyrophosphate-like adducts (phosphorylated methylphosphonates), which very slowly hydrolyzed following zero-order kinetics. This led to a complex mixture of phosphorus containing species with changing concentrations over time. We identified the molecular structure of these buffer adducts using 1D 1H-31P HSQC NMR and LC-ESI-MS/MS techniques. Reaction rates of adduct formation are fast enough to compete with hydrolysis in aqueous solution and to yield substantial amounts of buffer adduct over the course of just a couple of minutes. Possible reaction mechanisms are discussed with respect to the formation and subsequent hydrolysis of the pyrophosphate-like compounds as well as the increased rate of hydrolysis of the nerve agent to the corresponding alkyl methylphosphonates. In summary, the use of phosphate buffer for the development of new assays with sarin, soman and cyclosarin is discouraged. Already existing protocols should be carefully reexamined on an individual basis.     

Anti-TNF-alpha antibody (infliximab) therapy supports the recovery of eNOS and VEGFR2 protein expression in endothelial cells

The aim of this study is to investigate the effect of sera obtained from patients of Crohn's disease treated by anti-TNF-alpha antibody (Infliximab) on the expression of endothelial nitric oxide synthase (eNOS) and vascular endothelial growth factor receptor-2 (VEGFR2) protein in human umbilical vein endothelial cells (HUVEC) cultured in vitro. HUVEC was cultured in the presence of sera derived from patients before and after treatment, or from healthy individuals. Effects of sera on the expression of eNOS and VEGFR2 were monitored by determination of mRNA and protein levels using real time quantitative PCR and Western blot analysis, respectively. The serum of Crohn's patients contained elevated levels of TNF-alpha (34±1.80 pg/mL), which resulted in a decrease in the protein level of eNOS in HUVEC with a simultaneous induction of VEGFR2. Infliximab treatment normalized the expression level of these proteins by decreasing TNF-alpha level, particularly in those cases when clinical healing was also recorded, and it also conferred restitution of the level of angiogenic cytokines. Results suggest that altered angiogenesis possibly contributes to the initiation and perpetuation of inflammatory processes in inflammatory bowel disease (IBD). Endothelial dysfunction, a selective feature of Crohn's disease is beneficially affected by intravascular TNF-alpha neutralization.