层粘连蛋白、纤维粘连蛋白在胚胎发育中表达及作用

细胞外基质(ECM)主要分布于细胞外空间,包括胶原蛋白、非胶原蛋白和蛋白多糖三大类,在与其特殊黏附受体结合后,连接构成复杂的网架结构,起到调节组织发生和细胞生理活动的作用.层粘连蛋白(LN)、纤维粘连蛋白(FN)作为ECM的重要组成成分,广泛存在于各种动植物的细胞表面、细胞外液、结缔组织以及多数基底膜中.不仅在支持、连接和维持组织形态方面起重要作用,而且具有调节细胞黏附、生长、分化、促进细胞迁移、增殖、离子交换和信息传递的功能.对LN和FN的分子结构、合成分布及在胚胎着床、发育过程中表达与作用进行综述.     

A One-Stop Carpal Tunnel Clinic

INTRODUCTION

By December 2008, 90% of referrals requiring hospital admission will need to be seen and treated within the 18-week patient pathway. Previously, patients within our trust with suspected carpal tunnel syndrome had to wait 3 months to see a specialist in clinic and, once assessed, would have to wait up to a further 6 months for an open carpal tunnel decompression under local anaesthetic (OCTD/LA). We set up a one-stop clinic, where patients would have their out-patient consultation and surgery on the same day. We evaluated the clinic in order to assess whether it led to reduced waiting times whilst maintaining good clinical outcome and patient satisfaction.

PATIENTS AND METHODS

Patients were selected on the basis of the standard referral letter alone. Those selected were then assessed by a single surgeon in the clinic. The patients deemed appropriate underwent an OCTD/LA and were discharged the same day. Patients were followed up with a patient satisfaction and Boston questionnaire.

RESULTS

Forty-six patients underwent 63 OCTD/LA, waiting an average of 2.2 months (9 weeks) from referral. There was high patient satisfaction and improvement in symptoms following treatment in the clinic.

CONCLUSIONS

We believe a one-stop carpal tunnel clinic can be an efficient and cost-effective way of treating this common condition.     

实践表现的自我评估:美国内科委员会实践改善教育活动

为了评估美国内科委员会(American Board of Internal Medicine,ABIM)开展的实践改善单元(Practice Improvement Module,PIM)教育活动的效果,以及参与心脏疾病预防PIM教育活动的专科医师临床实践改善的表现,美国内科委员会进行了一项以自我指导为基础的观察研究.     

苯巴比妥负荷量防治新生儿缺氧缺血性脑病的用药时机

目的：分析苯巴比妥负荷量防治新生儿缺氧缺血性脑病的高效用药时机。方法：本研究选取某院2013-2016年间就诊的缺血缺氧性脑病（HIE）患儿169例进行研究，回顾性收集患儿治疗方案，并根据不同治疗方案将受试对象分为3组：A组患儿51例：24 h内使用负荷量苯巴比妥进行预防干预；B组患儿50例：24 h后使用苯巴比妥负荷量预防干预；C组患儿68例：未进行苯巴比妥负荷量预防，为对照组。收集患儿入院时基本情况，以及随访预防干预HIE的病情表现和预后状态，同时进行美国新生儿急性生理学评分国产期补充Ⅱ（SNAPPE-Ⅱ）的评分评价。讨论苯巴比妥负荷量防治新生儿缺氧缺血性脑病的最佳用药时机。研究中的数据均应用SPSS 20.0统计软件包进行分析。结果：本研究入院时3组患儿的SNAPPE-Ⅱ评分差别也无显著性差异。随访A组受试对象意识恢复最快，而C组最慢，P<0.05；反射和肌张力恢复时间也表现为A组最快而C组最慢，P<0.05；3组患儿随访SNAPPE-Ⅱ评分差别有显著性差异，P<0.05；A组评分水平最优，C组最差。同时统计患儿随访死亡发生情况，A组未检出死亡病例，B组和C组死亡率分别为2.00%和11.76%，C组岁最高，A组最低。结论：24 h内使用苯巴比妥负荷量防治新生儿缺氧缺血性脑病是一个高效的治疗时机，可有效改善患儿临床症状，并改善预后，减低死亡风险，值得临床推荐。     

Cook水囊在瘢痕子宫中期妊娠引产中的临床应用研究

目的探讨米索前列醇联合Cook水囊在瘢痕子宫中期妊娠引产中的应用价值。方法选择新疆医科大学第一附属医院计划生育病区剖宫产术后再孕、要求引产的患者50例。随机分为两组,对照组采用传统利凡诺引产,观察组采用米索前列醇联合Cook水囊引产。比较两组从规律宫缩至胎儿娩出时间、引产成功率、产后出血量、胎盘胎膜残留率及产道损伤情况。结果 (1)对照组从规律宫缩至胎儿娩出时间为(14.6±3.26)h,观察组从规律宫缩至胎儿娩出时间为(7.3±2.56)h,两组相比差异有统计学意义(P<0.05)。(2)对照组引产成功率为92%,观察组引产成功率为100%,两组相比差异无统计学意义(P>0.05)。(3)对照组产后24h出血量为(214±103)mL,观察组产后24h出血量为(156±98)mL,两组相比差异有统计学意义(P<0.05)。(4)对照组患者胎盘胎膜残留率为44%,引产成功者中有2例宫颈轻度裂伤。观察组胎盘胎膜残留率为24%,与对照组相比,观察组完全流产率高,清宫率低,引产并发症少,两组相比差异有统计学意义(P<0.05)。结论对于瘢痕子宫中期妊娠的患者,在米非司酮软化宫颈的基础上,联合米索前列醇和Cook水囊比单用依沙吖啶效果好,特别对于宫颈条件差、内口紧的患者,效果更佳。有引产成功率高、产后出血少、并发症少的优点。     

卵巢过度刺激综合征发病机制的研究进展

卵巢过度刺激综合征(OHSS)是促排卵过程中严重的医源性并发症,人绒毛膜促性腺激素诱导排卵使过多卵泡受刺激有关。其发病机制不明,病理生理特征主要为毛细血管通透性增加以及富含蛋白的液体停留在血管外间隙,导致血液浓缩,第三间隙水肿。现从血管内皮生长因子、血小板活化因子、溶血磷脂酸诱导炎性因子、囊性纤维化跨膜传导调节因子、肾素-血管紧张素系统对OHSS发病机制进行探讨分析,为临床更好地治疗和预防OHSS的发生提供理论依据。     

Identification of seven novel SMPD1 mutations causing Niemann–Pick disease types A and B

Niemann–Pick disease (NPD) types A and B are autosomal, recessively inherited, lysosomal storage disorders caused by deficient activity of acid sphingomyelinase (E.C. 3.1.4.12) because of mutations in the sphingomyelin phosphodiesterase‐1 (SMPD1) gene. Here, we present the molecular analysis and clinical characteristics of 15 NPD type A and B patients. Sequencing the SMDP1 gene revealed eight previously described mutations and seven novel mutations including four missense [c.682T>C (p.Cys228Arg), c.1159T>C (p.Cys387Arg), c.1474G>A (p.Gly492Ser), and c.1795C>T (p.Leu599Phe)], one frameshift [c.169delG (p.Ala57Leufs*20)] and two splicing (c.316+1G>T and c.1341delG). The most frequent mutations were p.Arg610del (21%) and p.Gly247Ser (12%). Two patients homozygous for p.Arg610del and initially classified as phenotype B showed different clinical manifestations. Patients homozygous for p.Leu599Phe had phenotype B, and those homozygous for c.1341delG or c.316+1G>T presented phenotype A. The present results provide new insight into genotype/phenotype correlations in NPD and emphasize the difficulty of classifying patients into types A and B, supporting the idea of a continuum between these two classic phenotypes.     

Methods for Examination an Explanted Coronary Sinus Lead Stabilized with a Coronary Stent

The aim of our investigation was to test the suitability of a novel method for the analysis of the integrity of an explanted pacemaker lead stabilized by a stent. A coronary sinus lead has been explanted 27 months after implantation and has been examined by optical‐, confocal‐, x‐ray‐, and scanning electron microscopy. Several surface injuries were found on the insulation. Based on the surface characteristics, it is possible to define and differentiate the source of damages as well as to measure the extent of injuries. Impedance of the explanted lead has also been measured and electronic integrity has been verified. (PACE 2013; 36:e27–e30)