76例重症肌无力患者临床治疗疗效分析

目的观察应用免疫球蛋白或血浆置换联合肾上腺皮质激素（ACS）治疗重症肌无力（MG）的疗效。方法对2010年1月至2012年1月76例重症肌无力病例的治疗方案进行回顾性分析：38例MG患者单纯应用大剂量甲基强的松龙短程冲击治疗,20例应用静脉注射大剂量免疫球蛋白（IVIg）联合肾上腺皮质激素治疗,18例应用血浆置换联合肾上腺皮质激素治疗。结果联合治疗组较ACS组治疗起效快,疗效维持时间长,且并发症少,联合治疗组治疗前后评分差值（P〈0．05）,有效率有统计学意义。结论采用静脉注射大剂量免疫球蛋白（IVIg）或血浆置换联合肾上腺皮质激素治疗效果优于单纯大剂量甲基强的松龙短程冲击治疗。     

大剂量免疫球蛋白、肾上腺皮质激素及血浆置换治疗重症肌无力疗效比较

目的探讨免疫球蛋白、血浆置换及肾上腺皮质激素(ACS)对重症肌无力(MG)的治疗效果。方法20例MG患者静脉注射大剂量免疫球蛋白(IVIg),23例应用肾上腺皮质激素治疗,22例应用血浆置换治疗。临床绝对评分及相对评分作为治疗前后疗效判定标准。结果65例MG患者治疗前后评分均有明显差异(P<0.05),ACS治疗前后评分差值较IVIg、血浆置换明显加大(P<0.05)。IVIg、血浆置换治疗MG起效快,但二者复发率及减效率明显高于ACS。结论从长期疗效看,ACS治疗MG效果优于IVIg、血浆置换。IVIg、血浆置换可作为MG的二线治疗方案及肌无力危象时的抢救方案。     

血浆置换法治疗重症肌无力患者的护理

采用血浆置换法治疗重症肌无力，在缓解肌无力症状方面，效果良好。笔者报告了９例重症肌无力患者７２次血浆置换治疗过程中的操作方法、术后常见并发症的防治措施以及出院指导。     

重症肌无力血浆置换中出现肌无力危象1例

治疗性血浆置换术(TPE)是采用血细胞分离机等方法去除患者含有致病因子的血浆，以达到缓解或减轻的目的。TPE广泛运用于血液系统、免疫系统和神经系统疾病等临床治疗。笔者在给1名重症肌无力患者做TPE过程中，患者出现症状加重，发生肌无力危象，报告如下。     

血浆置换法治疗重症肌无力患者的护理

采用血浆置换法治疗重症肌无力，在缓解肌无力症状方面，效果良好。笔者报告了９例重症肌无力患者７２次血浆置换治疗过程中的操作方法、术后常见并发症的防治措施以及出院指导。     

大剂量免疫球蛋白联合大剂量甲基强的松龙治疗重症肌无力临床观察

目的:探讨采用大剂量免疫球蛋白及大剂量甲基强的松龙治疗重症肌无力(MG)的临床疗效及安全性。方法:45例符合OssermanⅡb、Ⅲ型患者,随机分为观察组22例、对照组23例,观察组采用大剂量丙种球蛋白及大剂量甲基强的松龙治疗;对照组采用传统大剂量地塞米松静滴治疗。观察两组的临床绝对和相对评分、记录开始缓解时间和住院时间以及副作用。结果:观察组总有效率为86%,对照组为57%,两组相比差异有显著性(P<0.05);两组治疗前后临床绝对评分对比、开始缓解时间和住院时间比较均差异有显著性(P<0.05或P<0.01)。治疗过程中使用呼吸机,观察组2例,明显低于对照组的8例(P<0.05)。结论:采用大剂量免疫球蛋白及大剂量甲基强的松龙治疗Ⅱb或Ⅲ型MG较传统大剂量地塞米松治疗效果好,安全性高。     

血浆置换辅助治疗重症肌无力的临床疗效分析

目的评价血浆置换辅助治疗重症肌无力患者的临床效果。方法应用CS-3000PLUS全自动血细胞分离机对肌无力危象、为胸腺摘除术准备以及撤呼吸机困难患者进行血浆置换,以"706"代血浆和新鲜冰冻血浆作为置换液,每次置换血浆量为2 000 ml。结果 103名患者中每例进行PE 2～18次,平均(4.27±1.98)次,除1名肌无力危象患者因突发心肌梗塞死亡外,其余患者经过血浆置换单独或辅助治疗后均有显效或者好转,基本达到治疗目的。其中63名患者在93次血浆置换过程中出现不同程度副作用,包括口周麻木、四肢麻木、荨麻疹、寒战和发热等,经对症处理后均好转。结论血浆置换辅助治疗MG患者的肌无力危象、为胸腺摘除术准备以及撤呼吸机困难的1种有效治疗手段。     

血浆置换治疗伴呼吸肌无力危象的格林-巴利综合征和重症肌无力疗效观察

目的 观察血浆置换治疗伴呼吸肌无力危象的格林-巴利综合征（GBS）和重症肌无力（MG）患者的效果.方法 选择中山大学附属第一医院肾内科32例伴呼吸肌无力危象的GBS和MG患者中,15例采用单纯药物治疗,17例联用血浆置换,比较两组患者呼吸机辅助呼吸和住院时间.结果 血浆置换治疗组和单纯药物组呼吸机辅助呼吸时间分别为（8.7±8.4）d、（19.8±15.0）d,住院时间分别为（33.3±21.9）d、（52.7±28.4）d,两组比较差异有统计学意义（P＜0.05）.结论 血浆置换可有效缩短伴呼吸肌无力危象的GBS和MG患者呼吸机辅助呼吸和住院时间,值得临床推广应用.     

Maintenance plasma exchange treatment for muscle specific kinase antibody positive myasthenia gravis patients

Background:Anti‐muscle specific kinase antibody positive myasthenia gravis (MuSK MG) is often characterized by a relatively severe and progressive course, refractoriness to standard myasthenia gravis (MG) medications, and an increased risk of myasthenic crisis. We report here successful management of three MuSK MG patients using maintenance therapeutic plasma exchange (TPE) treatment for up to 4.5 years. Materials: The study was a 5‐year retrospective review of all MG patients treated with TPE between 2008 and 2013 at University of Michigan. Inclusion criteria of MuSK MG were positive for anti‐MuSK antibodies and a diagnosis of MuSK MG by staff neurologists. Patient data included age, gender, diagnostic testing results, medications, and the dates and response to TPE treatments. Results: A total of 153 MG patients underwent at least one course of TPE between 2008 and 2013. A total of 12 patients (7.8%) were positive for anti‐MuSK antibodies. Patients were predominantly female (83.3%) and a median age of onset was 46‐years old. Three MuSK MG patients were successfully managed with maintenance TPE. Conclusion: Maintenance TPE may be an effective option for MuSK MG patients. The key of successful maintenance treatment at our institution has been to tailor the TPE frequency for each individual, and to modify the treatment interval in conjunction with medical management. J. Clin. Apheresis 30:314–319, 2015. © 2014 Wiley Periodicals, Inc.     

血浆交换丙种球蛋白甲基强的松龙治疗重症肌无力

目的探讨依次采用少量血浆交换、大剂量丙种球蛋白、大剂量甲基强的松龙治疗重症肌无力 (MG)Ⅱ b或Ⅲ型的临床疗效及安全性.方法对符合 Osserman 分型为Ⅱ b或Ⅲ型的 37例 MG患者,按住院先后顺序随机分成两组.治疗组 18例,依次采用少量血浆交换、大剂量丙种球蛋白、大剂量甲基强的松龙治疗.对照组 19例,采用传统的大剂量地塞米松治疗.观察两组近期的临床疗效及安全性.结果治疗组症状缓解快,激素治疗过程中发生呼吸肌瘫痪、使用呼吸机现象少,住院时间短,死亡率低 (均 p<0.05).结论依次采用少量血浆交换、大剂量丙种球蛋白、大剂量甲基强的松龙治疗Ⅱ b或Ⅲ型 MG效果好,安全性高,值得临床推广应用.     

血浆置换在重症肌无力患者实施胸腺切除术前的应用与护理

目的探讨血浆置换在重症肌无力患者实施胸腺切除术中的应用。方法回顾性分析22例重症肌无力患者实施胸腺切除术前应用血浆置换的病例,总结护理要点。结果全部患者经血浆置换治疗后,肌无力症状明显好转,相关指标达到手术所需指征。结论重症肌无力患者实施胸腺切除术前应用血浆置换可及时改善患者的临床症状,使手术治疗更为安全有效,血浆置换过程中护理措施得当,可保证血浆置换治疗顺利进行。     

Prethymectomy plasmapheresis in myasthenia gravis

Plasma exchange before thymectomy may decrease the time on mechanical ventilation (MV) and shorten the stay in the intensive care unit (ICU) for patients with myasthenia gravis (MG). This study evaluated the effects of prethymectomy plasmapheresis. A total of 29 myasthenic patients, 18 women and 11 men aged 20-73 years, were treated with double filtration plasmapheresis (DFP) for two to five consecutive sessions over a period between 2 and 21 days (mean 8.1 days) before transsternal thymectomy. Acetylcholine receptor antibody (AchRAb) titers, vital capacity (VC), maximal inspiratory pressure (Pimax), and MG score were measured before and after the course of DFP. Three outcome measures including duration of postoperative hospital stay, duration of ICU stay, and duration of MV were analyzed for correlation with clinical variables. The duration of MV ranged from 6 to 93 h, with a median of 21 h. The median ICU stay was one day and the median postoperative hospital stay was 10 days. A higher removal rate of AchRAb was associated with a shorter duration of ICU and postoperative hospital stay (P = 0.001 and 0.019, respectively). Postoperative hospital stay was strongly correlated with post-DFP Pimax (P = 0.010), and marginally correlated with pre-DFP VC (P = 0.047) and to a lesser extent with pre-DFP Pimax (P = 0.063). Univariate analysis using the log rank test revealed that removal rate of AchRAb <30% (P = 0.043) and pre-DFP Pimax <-60 cmH2O (P = 0.024) were significantly associated with prolonged ICU stay. Risk factors for prolonged postoperative stay included post-DFP Pimax <-60 cmH2O (P = 0.017), pre-DFP Pimax <-60 cmH2O (P = 0.031), and post-DFP VC < 1.0 L (P = 0.046). Our results confirmed the efficacy and safety of DFP in prethymectomy preparation for myasthenic patients.     

胸腺瘤伴重症肌无力患者术后肌无力危象的临床分析

目的：探讨胸腺瘤伴重症肌无力（MG）患者行胸腺瘤切除术后,发生肌尢力危象的影响因素及治疗措施。方法：48例胸腺瘤伴MG患者行扩大胸腺切除术,术后是否发生肌无力危象分组。分析肌无力危象与性别、年龄、病史、Osserman分型、胸腺瘤病理类型、Masaoka病理分期及术前用药关系,建立人工气道与机械通气治疗肌无力危象疗效。结果：18例患者于术后（1．69±1．62）d发生肌无力危象,肌无力危象与年龄、性别、Osser—msn分型、病理类型、Masaoka分期及术前用药无关。9例气管切开、6例鼻腔气管插管、3例口腔气管插管．机械通气（12．0±13．88）d,肌无力危象组住院时间明显延长（34．61±23．90d vs 20．33±15．26d,P〈0．05）,10例给予大剂量激素冲击治疗,其中1例发生双侧股骨头坏死,无一例死亡。结论：胸腺瘤伴MG患者术后肌无力危象的发生与胸腺瘤关系不密切,迅速建立人工气道与合理的机械通气策略是救治肌无力危象的关键。     

Clinical evaluation of a staphylococcal protein A immunoadsorption system in the treatment of myasthenia gravis patients

BACKGROUND: The role of plasma exchange is well established in the management of myasthenia gravis, an autoimmune disorder characterized by muscle weakness and caused by circulating IgG antibodies with specificity against the acetylcholine receptor. Plasma antibody removal by conventional means, however, is nonselective and uses replacement fluids (chiefly, albumin solution) derived from human plasma. STUDY DESIGN AND METHODS: The Canadian Apheresis Group undertook a study at two Canadian apheresis centers to clinically evaluate a staphylococcal protein A immunoadsorption system (EXCORIM) in myasthenia gravis patients. RESULTS: The immunoadsorption system was safe and well tolerated. Ten of 12 patients had improvement in their neurologic status, as measured by a 20-point scoring system. The mean improvement in the weakness score was significant for the group (p = 0.0013). CONCLUSION: Patients with myasthenia gravis respond to treatment with plasma immunoadsorption. Further studies are required for a cost-benefit analysis.     

Laparoscopic assisted colectomy and myasthenia gravis

Summary

Myasthenia gravis is an auto-immune disorder posing particular challenges in the anaesthesia of patients requiring laparotomy. This report describes how a laparoscopic approach may overcome these difficulties.     

胸腺瘤病理与重症肌无力关系的临床研究

目的进行胸腺瘤分类标准的临床研究,以探讨胸腺瘤最新 WHO病理分型与重症肌无力( MG)发生率、 Osserman分型及手术预后的关系.方法回顾分析 1974/2000 105例因胸腺瘤行胸腺切除的患者,分别应用胸腺瘤的传统病理分类法、 Levine Rosai分类法及最新 WHO分型标准对胸腺瘤分类,并在 MG发生率、 Osserman分型及手术预后等方面统计比较.结果 ( 1) A型+ AB型良性例数较多, B型恶性例数较多,体现出 A型及 AB型胸腺瘤良性的特点.( 2) B3型较 A型及 AB型易合并 MG(χ 2 = 3.294 8, P= 0.07), C型 13例均未合并 MG. B3型胸腺瘤合并 MG的手术危象发生率比 A型+ AB型、 B1+ B2型高,但统计学上无显著差异.( 3)手术危象与 Masaoka分期的良、恶性程度明显相关(χ 2= 4.218 8, P= 0.04) ,主要集中在 Osserman改良分型Ⅱ b型及Ⅲ型(χ 2= 13.099 4, P< 0.001).结论胸腺瘤最新 WHO病理分型对于区别良恶性肿瘤有指导意义;不同类型的胸腺瘤 MG易患性不同,并且结合 Osserman临床分型、 Masaoka病理分期对提示术后危象有一定的应用价值.     

Eculizumab for the treatment of myasthenia gravis

ABSTRACT

Introduction

Acetylcholine receptor antibody-positive generalized myasthenia gravis (gMG) is effectively treated with symptomatic and immunosuppressive drugs but a proportion of patients has a persistent disease and severe adverse events (AEs). The unmet medical needs are specific immunosuppression and AE lowering. Eculizumab blocks C5 protecting neuromuscular junction from the destructive autoantibody effects. Phase II (Study C08-001) and III (ECU-MG-301) studies, with the open-label extension (ECU-MG-302), demonstrated eculizumab efficacy and safety in refractory gMG patients.