免疫吸附治疗重症肌无力术后肌无力危象

重症肌无力(myasthenia gravis,MG)是累及神经一肌肉接头突触后膜上乙酰胆碱受体(AchR).主要由AchR抗体(AchR-Ab)介导的的自身免疫性疾病[1].目前胸腺切除已经成为治疗MG的重要治疗方法.肌无力危象是胸腺切除术后最严重的并发症,病情危急,死亡率高田.南华大学附属第二医院心胸外科自2001年至2006年应用葡萄球菌蛋白A(staphylococcal protein A,SPA)免疫吸附治疗MG术后肌无力危象患者11例,取得了满意的疗效,现总结报道如下.     

218例重症肌无力危象的临床分析

目的总结重症肌无力危象的临床特点及急救和预防经验.方法回顾性分析1956～2004年诊治的218例患者369例次重症肌无力危象的病例资料.结果死亡51例,病死率23.39%;近十年(1994～2004年)死亡4人,病死率4.2%.结论综合利用气管切开正压辅助呼吸、激素冲击治疗、丙种球蛋白静滴及血浆交换可改善重症肌无力危象的预后,且明显降低病死率.     

重症肌无力危象的临床护理

重症肌无力（MG）是神经肌肉部位传递障碍引起的一种自身免疫性疾病,病变主要累及神经肌肉接头后膜上乙酰胆碱受体,主要症状为部分或全身骨骼肌极易疲劳,通常在晨轻晚重,活动后加剧,经过休息和胆碱酯酶抑制剂治疗后有所恢复。肌无力危象是MG病人突然发生呼吸肌无力,致呼吸极度困难,难以吞咽、不能维持基本生活和生命体征的危急状态,其病死率为15．4％～50．0％,常见诱因为强烈的精神刺激、劳累、停药、感染等,若抢救不及时可加重病情,增加医疗费用,或因窒息及发生呼吸功能衰竭而死亡,     

重症肌无力169例临床分析

目的研究重症肌无力(MG)临床特点、误诊原因与MG危象临床表现。方法分析本院收治的169例MG患者的临床资料。结果本文患者均行新斯的明试验,阳性率100%。85例行重复神经电刺激(RNS)检查,64例低频波幅递减,8例同时高频波幅递减。157例行胸腺影像学检查,发现胸腺异常80例。发生肌无力危象31例次。121例行甲状腺功能(FT3、FT4、TSH)检测,发现甲状腺功能异常17例。结论MG临床表现多样,时有误诊,易于合并胸腺瘤或胸腺增生及甲状腺功能异常。肌无力危象发生率高。     

血浆交换治疗重症肌无力危象28例临床分析

目的 评估血浆交换（PE）疗法抢救重症肌无力危象的有效性安全性。方法 28例患者于确诊当日进行PE,隔日一次,四次为一疗程,观察疗效和副作用。结果 血浆交换虽有低血压、低血钾、低血钙等副作用,但对症处理即可缓解,总有效率89．3％。结论 PE是抢救重症肌无力危象的一种安全有效的治疗手段,采用必要的预防措施,可避免副作用的产生。     

27例重症肌无力危象抢救分析

目的复习重症肌无力（MG）危象的抢救体会及经验。方法对近5年抢救27例MG危象的临床特点、抢救措施进行总结分析。结果平均发病年龄约41岁，感染为常见诱因，气管切开，呼吸机辅助呼吸，静脉注射大剂量免疫球蛋白（IVIg）及肾上腺皮质激素（ACS）冲击治疗，预防感染为主等抢救治疗方法，无1例死亡。结论及时插管至气管切开。应用呼吸机，预防感染，肾上腺皮质激素冲击治疗，静脉注射大剂量免疫球蛋白（IVIg）等综合抢救措施，明显缩短了病程，显著降低了死亡率。     

重症肌无力伴胸腺瘤患者术后危象的原因分析

我们对9例伴有胸腺瘤的重症肌无力(MG)术后发生危象的患者,分析其原因并探讨其救治方法.     

27例重症肌无力危象抢救分析

目的复习重症肌无力(MG)危象的抢救体会及经验。方法对近5年抢救27例MG危象的临床特点、抢救措施进行总结分析。结果平均发病年龄约41岁,感染为常见诱因,气管切开,呼吸机辅助呼吸,静脉注射大剂量免疫球蛋白(IVIg)及肾上腺皮质激素(ACS)冲击治疗,预防感染为主等抢救治疗方法,无1例死亡。结论及时插管至气管切开,应用呼吸机,预防感染,肾上腺皮质激素冲击治疗,静脉注射大剂量免疫球蛋白(IVIg)等综合抢救措施,明显缩短了病程,显著降低了死亡率。     

重症肌无力病人危象82例临床分析

目的 探讨重症肌无力（MG）危象的诱发因素、延长气管插管的因素以及并发症、病死率。方法 回顾性分析了82例MG病人（87次危象）的年龄、性别、发病年龄、诱发因素、气管插管并发症及影响插管时间的因素。结果 75％病人存在诱发危象的因素，其中感染为常见因素，其次外伤、劳累、月经及分娩，医源性因素占24％。有9个因素延长气管插管。12例死亡，病死率为14％。结论 积极治疗及预防肌无力危象病人气管切开后并发症，可有效缩短气管插管的时间，降低病死率。     

Clinical characteristics and predictive factors of myasthenic crisis after thymectomy

The aims of this study were to assess the clinical characteristics of myasthenic crisis after thymectomy (MCAT) and to identify predictors affecting the occurrence of MCAT. Of 66 patients with myasthenia gravis (MG), MCAT occurred in 20 patients (30.3%). The median time interval from thymectomy to MCAT was 3.4 months. MCAT occurred in 65.0% of patients within the first 6 months of a thymectomy, and 35.0% after 6 months. A second MCAT occurred in nine (45.0%) patients who survived the first MCAT, and in seven (50.0%) of 14 patients with a history of a preoperative myasthenic crisis before thymectomy (MCBT). A history of MCBT, and clinical factors reflecting perioperative clinical severity at thymectomy, including preoperative Osserman’s grade, bulbar symptoms, use of immunosuppressants, pulmonary function, and postoperative delayed ventilator weaning, were significantly correlated with the occurrence of MCAT on univariate analysis. However, a history of MCBT was the only independent factor affecting the occurrence of MCAT on multivariate logistic regression analysis (odds ratio, 17.9; 95% confidence interval, 4.019-79.873; p < 0.001). Thus, the occurrence of MCAT may be correlated only with a history of MCBT rather than with factors reflecting perioperative clinical severity. MG patients with a history of MCBT are more susceptible to MCAT, particularly within the first 6 months of thymectomy.     

Noninvasive ventilation in myasthenic crisis

BACKGROUND: Myasthenic crisis (MC) is often associated with prolonged intubation and with respiratory complications. OBJECTIVES: To assess predictors of ventilation duration and to compare the effectiveness of endotracheal intubation and mechanical ventilation (ET-MV) with bilevel positive airway pressure (BiPAP) noninvasive ventilation in MC. DESIGN: Retrospective cohort study. SETTING: Academic research. Patients We reviewed consecutive episodes of MC treated at the Mayo Clinic, Rochester, Minnesota. MAIN OUTCOME MEASURES: Collected information included patients' demographic data, immunotherapy, medical complications, mechanical ventilation duration, and hospital lengths of stay, as well as baseline and preventilation measurements of forced vital capacity, maximal inspiratory and expiratory pressures, and arterial blood gases. RESULTS: We identified 60 episodes of MC in 52 patients. BiPAP was the initial method of ventilatory support in 24 episodes and ET-MV was performed in 36 episodes. There were no differences in patient demographics or in baseline respiratory variables and arterial gases between the groups of episodes initially treated using BiPAP vs ET-MV. In 14 episodes treated using BiPAP, intubation was avoided. The mean duration of BiPAP in these patients was 4.3 days. The only predictor of BiPAP failure (ie, requirement for intubation) was a Pco(2) level exceeding 45 mm Hg on BiPAP initiation (P= .04). The mean ventilation duration was 10.4 days. Longer ventilation duration was associated with intubation (P= .02), atelectasis (P< .005), and lower maximal expiratory pressure on arrival (P= .02). The intensive care unit and hospital lengths of stay statistically significantly increased with ventilation duration (P< .001 for both). The only variable associated with decreased ventilation duration was initial BiPAP treatment (P< .007). CONCLUSIONS: BiPAP is effective for the treatment of acute respiratory failure in patients with myasthenia gravis. A BiPAP trial before the development of hypercapnia can prevent intubation and prolonged ventilation, reducing pulmonary complications and lengths of intensive care unit and hospital stay.     

Predictors of outcome of myasthenic crisis

There is paucity of study on predictors of myasthenic crisis (MC), prolonged ventilation and their outcome, a reason why this study was undertaken. Sixty-four patients with myasthenia gravis (MG) were included whose median age was 45 (6–84) years. Their clinical treatment, presence of thymoma, anti-acetylcholine receptor antibody (AchRAb), thymectomy, comorbidities, offending drugs and occurrence of MC were noted. Patients needing prolonged ventilation (>15 days) were noted. Hospital mortality, MG quality of life (QOL) at discharge and thereafter annual hospital visit, admission, expenditure and work day loss were enquired. Fourteen (21.9 %) patients had MC within 1–120 (median 8.5) months of disease onset within a median follow-up of 48 (3–264) months. The precipitating factors were infection in six, surgery in five, tapering of drugs in two and reaction to iodinated contrast in one patient. Male gender, bulbar weakness, AchRAb, thymoma, surgery and comorbid illnesses were related to MC. Eight of them (57.1 %) needed prolonged ventilation. Half the patients with MC had recurrent crisis (2–4 attacks). Death was not related to MC although MC patients had worse QOL, higher annual treatment expenditure with frequent hospital visit and hospitalization. In conclusion, association of comorbid illness with MC and prolonged ventilation highlights the need of close follow-up and appropriate management.