Dominantly inherited tubular aggregate myopathy.

We report an unusual familial myopathy characterized morphologically by the presence of large tubular aggregates in all fibre types. Two patients, a father and daughter, presented with slowly progressive proximal weakness, limitation of eye movement, and Achilles tendon contractures. Serum creatine kinase was 5-10 times normal. Light microscopy revealed type I fibre predominance. Basophilic accumulations, which stained intensely with the NADH-TR reaction, were present in both fibre types. Electron microscopy revealed that these consisted of tightly packed parallel tubular arrays. These varied somewhat in their ultrastructural appearance and were classified accordingly as type I, II, and III tubular structures. The tubular aggregates appear to be derived from the sarcoplasmic reticulum. This report further supports the evidence of a distinct clinico-pathological entity of genetic origin.     

Acute quadriparesis in an asthmatic treated with atracurium

An 18-yr-old male asthmatic was paralyzed with atracurium for a period of seven days to facilitate mechanical pulmonary ventilation. After withdrawal of the muscle relaxant, train-of-four neuromuscular monitoring demonstrated rapid recovery of normal function. Three days later he developed acute quadriparesis without respiratory compromise. Electrophysiological studies showed normal conduction velocities, low compound muscle action potential amplitudes and evidence of denervation. Most cases of post-ventilatory weakness in the ICU involve the use of vecuronium and pancuronium. It has been suggested that the steroid nucleus in these muscle relaxants may be responsible. Our patient developed generalised weakness after treatment with atracurium, a benzylisoquinolinium muscle relaxant. Thus, it appears that the steroid nucleus of vecuronium and pancuronium is not essential in causing post-ventilatory weakness.     

System weaknesses as contributing causes of accidents in health care.

OBJECTIVE: Accidents in health care, resulting in injury or death to the patient, are a matter of considerable concern. The aim of this study is to examine whether system weaknesses can contribute to these accidents, and if so, how. DESIGN: Eight consecutive accidents reported to the Health Authority in Sweden were analysed using MTO (Man-Technique-Organization) analysis. SETTING: Emergency care hospitals in Sweden. RESULTS: All cases that involved the system supported the assumption that system weaknesses are a contributing factor to accidents. In this study two types of latent failure could be identified: process control latent failures and interactional latent failures. The time span from activation of process control latent failures to operator error was very short, and the study demonstrates the simple relationship between situational factors and operator errors. Interactional latent failures exert system influence in a more indistinct manner. Latent failures, as seen in this study, act not only by creating opportunities for operator errors but also by hindering error detection in the time window available. Safety barriers, which might have prevented the accidents, could be proposed in seven out of eight cases. CONCLUSION: System weaknesses seem to play an important role in accident evolution. Consequently, certain measures can be suggested in order to improve patient safety: (i) sufficient resources should be allocated for research and development at both medical schools and hospitals in order to establish competence and procedures for systematic analyses of processes; and (ii) authorities handling accident cases should have adequate competence in system analysis.     

持续胸痛4个月及进行性下肢无力2个月

患儿,男,5岁。因胸痛4个月,右下肢无力2个月,双下肢无力10 d入院,无排便、排尿障碍,无意识障碍,双下肢呈上运动神经元性瘫痪,无颅神经受累,无感觉障碍。脊髓MRI示颈6~胸2椎管内肿瘤,压迫脊髓。转入神经外科手术治疗,患儿手术切除肿瘤后逐渐康复,随访6年未复发。该患儿病理诊断为透明细胞型脑（脊）膜瘤（WHO Ⅱ级）。儿童胸痛伴运动障碍,应与脊膜瘤这种椎管内肿瘤鉴别。     

脾虚泄泻患者肠粘膜中SP和VIP初步探讨

本研究用放射免疫技术对30例脾虚泄泻患者回肠末端、横结肠和乙状结肠粘膜中 P 物质(SP)和血管活性肠肽(VIP)进行测定,发现脾虚泄泻组回肠末端 SP 含量(120.95±90.70pg/mg 湿重)较无腹泻组(47.86±35.49pg/mg 湿重)和非脾虚泄泻组(52.50±42.49pg/mg 湿重)显著增加;脾虚泄泻组乙状结肠粘膜 VIP 含量(510.63±265.22pg/mg 湿重)较无腹泻组(308.67±204.49pg/mg 湿重)也显著增加;与非脾虚泄泻组比较(398.97±240.80pg/mg 湿重)有增加趋势,但无统计学差异,提示 SP 和 VIP 可能与脾虚泄泻的腹泻症状有关。根据脾虚临床表现和 VIP 的功能,脾虚泄泻中 VIP 可能起更重要作用。     

肌肉超声回声联合血浆炎性因子对ICUAW诊断及预后评估的价值研究

目的 探讨重症监护病房（intensive care unit,ICU）获得性肌无力（ICU acquired weakness,ICUAW）患者肌肉超声回声与血浆炎性因子的相关性,以及其对ICUAW的诊断价值和预后的预测价值。方法 选择重庆市急救医疗中心ICU住院患者,分别在第1、3、7天使用床旁超声检测患者肌肉回声,获得的总体肌肉回声评分（global muscle echogenicity score,GEM）,测定血清白细胞介素-6（interleukin-6,IL-6）和降钙素原（procalcitonin,PCT）浓度,采用医学研究理事会肌力评分法（medical research council scales,MRC-ss）评估肌肉力量。根据患者入ICU第7天MRC-ss评分将患者分为ICUAW组和非ICUAW组,分析比较2组患者GEM、IL-6、PCT的差异及各指标的相关性。利用受试者工作特征（receiver operator characteristic,ROC）曲线分析以上参数对ICUAW诊断效能,分析GEM、IL-6、PCT对ICUAW患者的预测预后价值。结果 ICUAW组第3天GEM、第7天IL-6浓度、GEM高于非ICUAW组（P<0.05）。GEM与第7天IL-6水平呈正相关（r=0.221）,第7天GEM与MRC-ss评分呈负相关（r=-0.581）。ROC曲线分析显示,第7天GEM对ICUAW有诊断预测价值,ROC曲线下面积（area under the curve,AUC）为0.838,使用GEM、IL-6、PCT联合诊断,AUC=0.885（P<0.05）。ICUAW组Barthel指数评分（Barthel index,BI）低于非ICUAW组,ICUAW组中总体肌肉超声回声评分（global muscle echogenicity score,GEM）高的患者BI低于GEM低的患者（P<0.05）。结论 ICU住院患者GEM与IL-6、PCT浓度相关,其对ICUAW具有一定的诊断价值,并能够预测ICUAW患者的预后。     

Effect of early progressive mobilization on intensive care unit-acquired weakness in mechanically ventilated patients: An observational study

Early progressive mobilization is usually considered as an effective method for intensive care unit-acquired weakness (ICU-AW), but the controversies on this topic remain debatable, especially in initiation time, safety profile, and other implementation details. So, more studies should be performed to solve these disputes. A set of critically ill patients underwent mechanical ventilation in intensive care unit (ICU) of our hospital from March 2018 to September 2020 were included as study object. Patients received early progressive mobilization were included into the intervention group (n = 160), and another patients matched with the intervention group by gender, age, and APACHE II score, and these patients received routine intervention were included into the control group (n = 160). Then, indexes involving muscle strength, Barthel index, functional independence, incidence rates of ICU-AW and other complications were comparatively analyzed between the 2 groups. The Medical Research Council score and Barthel index score in the intervention group were significantly higher than those in the control group (all P < .05). The percentages of patients who were able to complete taking a shower, wearing clothes, eating, grooming, moving from bed to chair and using the toilet by alone in the intervention group were significantly higher than those in the control group (69.38% vs 49.38%, 73.13% vs 51.88%, 81.25% vs 55.63%, 74.38% vs 48.75%, 82.50% vs 65.63%, 78.13% vs 63.13%, respectively, all P < .05). The incidence rate of ICU-AW and overall incidence rate of complications in the intervention group were significantly lower than those in the control group (6.88% vs 28.13% and 23.13% vs 48.13%, both P < .05). Early progressive mobilization can effectively increase muscle strength and daily basic motion ability, improve functional status, and decrease risk of ICU-AW in critically ill patients underwent mechanical ventilation, and it has an attractive application value in clinic.     

不同频率神经肌肉电刺激对ARDS相关性ICU-AW小鼠肌肉萎缩防治及临床意义

目的 不同频率下神经肌肉电刺激(neuromuscular electrical stimulation,NMES)在ARDS相关性ICU获得性衰弱(ICU-acquired weakness,ICU-AW)中的作用及机制。方法 健康雄性C57BL/6小鼠88只随机分为11组,每组8只。分为:空白对照组(C1)、气管内注入无菌水组(C2)、ICU-AW模型组(ICU-AW)、ICU-AW+AMPK激动剂A-769662组(ICU-AW-A)、ICU-AW+A-769662溶剂对照组(ICU-AWV)、NMES 20 Hz组(ICU-AW-20)、NMES 40 Hz组(ICU-AW-40)、NMES 60 Hz组(ICU-AW-60)、NMES80 Hz组(ICU-AW-80)、ICU-AW-40+AMPK抑制剂Compound C组(ICU-AW-40-C)、ICU-AW-40+Compound C溶剂对照组(ICU-AW-40-V)。检测小鼠四肢抓力和存活状态,7 d后收集小鼠肺组织和腓肠肌标本,采用HE染色观察肺和肌肉病理学变化,采用western blot以及qRT-PCR的方...     

从壮医“毒虚致病”探讨慢性肾脏病

水道疾病的病机主要为外感邪毒或邪毒内生、身体虚弱、过度劳累影响肾和膀胱功能,毒邪内阻使水道不畅,气机受阻,功能失调,或正气亏虚,对水道及其脏腑功能的调节能力下降,使天、地、人三气不能同步而发病。壮医认为毒虚致百病,毒、虚是慢性肾脏病致病的因素,两者贯穿于慢性肾脏病的整个疾病过程。运用壮医特色"三道两路""三气同步"的基本理论与"毒虚致百病"的病因病机,通过调气、解毒、补虚原则指导治疗慢性肾脏病。     

康欣胶囊及其拆方对血管性痴呆肾虚血瘀型模型大鼠外周血CD3、CD4、CD8和CD4/CD8的影响

目的 　观察康欣胶囊及其拆方对 ( VD)模型 (肾虚血瘀型 )大鼠外周血 CD3、CD4、CD8的影响。 方法 　采用电灼烧封闭双侧椎动脉和摘除单侧性器官法制作 ( VD)模型 (肾虚血瘀型 )大鼠 ,用康欣胶囊全方及其拆方和西药喜得镇给药 1个月 ,电镜、光镜观察各组大鼠海马、下丘脑、枕部皮质神经细胞情况 ,流式细胞仪检测大鼠外周血 CD3、CD4、CD8。 结果 　康欣胶囊全方组海马、下丘脑、枕部皮质神经细胞的内织网、线粒体水肿固缩及细胞核固缩情况明显轻于模型空白对照组 ;外周血 CD4/ CD8比例指标明显高子空白模型组、健脾组、西药组 ( P<0 .0 1) ,养血活血组 CD4/ CD8比例指标高于空白模型组、健脾组 ( P<0 .0 5 )。结论 　康欣胶囊全方及其拆方养血活血方可提高 ( VD)模型 (肾虚血瘀型 )大鼠外周血 CD4/ CD8比例水平即提高免疫力 ,并延缓减轻缺血性神经细胞凋亡。