重症肌无力患者血清titin抗体

目的 探讨血清抗titin抗体对重症肌无力合并胸腺瘤诊断的临床意义。方法 用ELISA法分别测定正常对照、重症肌无力、重症肌无力合并胸腺瘤患者及非重症肌无力胸腺瘤患者血清titin抗体及乙酰胆碱受体抗体,并比较其临床意义。结果 与重症肌无力患者相比,重症肌无力合并胸腺瘤患者的血清titin抗体阳性率明显增高,分别为3％和94％,且titin抗体阳性的重症肌无力病情较重。非重症肌无力胸腺瘤患者血清中亦可检出titin抗体。结论 titin抗体检查对于重症肌无力合并胸腺瘤的诊断及判定预后具有重要临床意义。     

重症肌无力患者血清Titin抗体和RyR抗体的临床研究

目的探讨重症肌无力（MG）患者血清Titin抗体和Ryanodine受体（RyR）抗体的临床意义。方法采用ELISA法检测61例MG患者和35例健康体验者（正常对照组）的血清Titin抗体、RyR抗体、AChR抗体。结果MG组Titin抗体和RyR抗体阳性率均显著高于对照组（均P＜0.001）;合并胸腺瘤MG（MGT）组患者Titin抗体阳性率明显高于未合并胸腺瘤MG（NTMG）组（P＜0.01）;MGT组和NTMG组患者RyR抗体阳性率差异无统计学意义（P＞0.05）;晚发型MG患者中Titin抗体和RyR抗体阳性率均明显高于早发型MG患者（均P＜0.05）;全身型或重症型（ⅡA、ⅡB、Ⅲ、Ⅳ型）Titin抗体阳性率显著高于眼肌型（Ⅰ型）（P＜0.01）,而两组间RyR抗体阳性率未见统计学差异（P＞0.05）;MG患者Titin抗体和RyR抗体水平与肌无力严重程度相关分析呈正相关（r=0.520、0.487,均P＜0.01）。结论Titin抗体和RyR抗体检测有助于MG的诊断,且Titin抗体有助于鉴别MG患者是否伴随胸腺瘤及进行Osserman分型。Titin抗体与RyR抗体水平还与MG患者病情的严重程度相关,可应用于评估MG患者的病情与预后。     

重症肌无力患者血清乙酰胆碱受体抗体与柠檬酸提取物抗体测定及其临床意义

研究重症肌无力（ＭＧ）病人血清乙酰胆碱受体抗体（ＡｃｈＲａｂ）与抗骨骼肌柠檬酸提取物抗体（ＣＡＥａｂ）的含量改变及临床意义。方法以固相酶联免疫吸附法测定１２３例ＭＧ病人血清ＡｃｈＲａｂ与ＣＡＥａｂ含量。结果两种抗体的阳性率分别为７８％和２４．４％，明显高于健康献血员和非ＭＧ病人组（Ｐ＜０．０１）。ＣＡＥａｂ在合并胸腺瘤组与临床无瘤组的阳性率分别为７１％和９．８％，差别有显著性（Ｐ＜０．０１）。结论ＡｃｈＲａｂ的测定可为ＭＧ病人的早期诊断提供可靠的实验室依据，ＣＡＥａｂ的测定有助于合并胸腺瘤ＭＧ病人的早期发现。     

重症肌无力患者的抗横纹肌抗体测定

100例重症肌无力患者血清中抗横纹肌抗体阳性率为47%。伴胸腺肿瘤的重症肌无力患者其阳性率为80%,不伴胸腺瘤者仅为45%,抗横纹肌抗体阴性则伴胸腺瘤的可能性较小。     

小儿重症肌无力血清乙酰胆碱受体抗体的测定及其临床意义

为研究小儿重症肌无力(MG)血清乙酰胆碱受体抗体(AchRab)的含量改变及其临床意义。采用固相酶联免疫吸附法检测血清AchRab的含量。结果MG患儿血清AchRab阳性率为81%,明显高于健康对照组和非MG病人组(P<0.01)。MG患儿治疗后血清AchRab的含量明显低于治疗前,其阳性率分别为55%和81%(P<0.05)。结果提示AchRab的测定可作为MG患儿早期诊断和疗效观察的指标。     

重症肌无力合并胸腺瘤的CT及血清相关抗体

目的探讨胸腺瘤相关抗体与CT检查对重症肌无力合并胸腺瘤诊断的临床意义。方法对32例胸腺CT检查异常并行手术治疗的重症肌无力患者的血清titin和兰尼定碱受体(RyR)抗体进行检测,并与术后病理诊断进行比较。结果32例患者经术后病理检查,21例为胸腺瘤、11例为胸腺增生;CT检查敏感性为90·5%;CT结合胸腺瘤相关抗体检查对胸腺瘤诊断的特异性为100%。结论血清胸腺瘤相关抗体检查有助于胸腺瘤及胸腺增生的鉴别诊断,如与CT相结合,能提高其诊断胸腺瘤敏感性。     

重症肌无力相关自身抗体的研究进展

重症肌无力（MG）是由自身抗体引起、T细胞免疫依赖、补体参与的主要累及神经肌肉接头处的自身免疫性疾病.乙酰胆碱受体抗体（AchR-Ab）在MG中的阳性率为85% ～90%,对MG的诊断具有重要意义.但研究中发现,MG患者血清中还存在其他自身抗体,如肌联蛋白抗体（Titin-Ab）对胸腺瘤合并MG（MGT）的诊断也具有良好的灵敏性;酪氨酸激酶抗体（MuSK-Ab）主要存在于AchR-Ab阴性的MG患者血清中;突触前膜抗体（PsM-Ab）能反映突触前膜的损坏与AchR-Ab水平高度相关;兰尼碱受体抗体（RyR-Ab）对诊断MGT的特异性高,在迟发型MG中阳性率高.     

ABC-ELISA测定重症肌无力患者血清抗乙酰胆碱受体抗体水平的研究

采用ABC-ELIsA法检测了50例重症肌无力(MG)患者,20例其它中枢神经系统(CNS)疾病患者及30例正常人血清。发现:临床对照及正常对照组乙酰胆硷受体抗体(AchRAb)均为阴性。50例MG中有21例阳性(42%),全身型MG阳性率明显高于单纯眼肌型(P<0.005),随着病程延长,病情好转,大多患者的AchR抗体OD值呈下降转阴趋势。个别病例抗体比值与临床不符,可能是由于AchRAb的异质性所造成。ABC-ELISA法简便、快速可靠,为MG深入研究提供了有效手段。     

血清连接素抗体水平在重症肌无力伴胸腺异常中的临床意义

目的探讨连接素抗体(Titin Ab)在重症肌无力(MG)伴胸腺异常患者中的临床意义.方法本院112例MG患者,根据病理检查确定胸腺增生和胸腺瘤的诊断,并根据胸腺病理结果分为3组,即MG伴胸腺瘤(MGT)组、MG伴胸腺增生(MGH)组及胸腺正常(MGN)组,采用ABC-ELISA法测定血清乙酰胆碱受体抗体(AChR Ab)水平和ELISA法测定血清Titin Ab水平,进行比较分析.结果(1)MGT组Titin Ab阳性率为72.9%,明显高于MGH组的12.5%和MGN组的22.2%(均P<0.05);(2)血清Titin Ab阳性者发病年龄、病情严重程度评分及血清AChR Ab水平均高于血清Titin Ab阴性者(均P<0.01);(3)MG患者血清Titin Ab水平与病情严重程度评分、血清AChR Ab水平呈正相关(均P<0.01);(4)随访发现Titin Ab阳性者术后1.5年内症状再度加重9例12次,中位复发时间10个月,明显高于Titin Ab阴性者的1例次(P<0.05).结论血清Titin Ab水平可能是MGT患者临床诊断及预后判断的一个新指标.     

重症肌无力患者血清抗乙酰胆碱受体抗体和抗突触前膜抗体检测

目的:探讨重症肌无力(MG)患者血清抗乙酰胆碱受体抗体(AchRab)和抗突触前膜抗体(Psmab)水平与其临床特征之间的关系.方法:采用ABC-ELISA法检测186例MG患者及35例正常人血清AchRab和Psmab.结果:MG患者血清AchRab和Psmab水平及阳性率均高于对照组(P＜0.01),且2抗体水平高度相关(r=0.72,P＜0.001).2抗体水平与MG患者的性别无关;1～14岁患者2抗体水平较低;胸腺瘤患者2抗体水平高于胸腺增生者;危象型患者高于轻型和混合型患者;手术摘除胸腺后,2抗体水平较术前下降(P均＜0.05).结论:AchRab和Psmab与MG患者病情程度及病理类型有关,可用于MG的病情监测.     

重症肌无力患者血清中多种抗体的检测及意义

Background Antibodies against acetylcholine receptor, acetylcholinesterase, ryanodine receptor and titin have been found in patients with myasthenia gravis. However, the relations between these antibodies and character of myasthenia gravis are unknown. This study aimed to detect multiple antibodies in myasthenia gravis and to investigate its clinical significance. Methods These antibodies were detected by enzyme-linked immunoabsorbent assay in 89 cases of myasthenia gravis, 66 cases of other neurological diseases and 66 healthy controls. The incidences of antibodies were compared using the chi-square test. Results Acetylcholine receptor, acetylcholinesterase, titin and ryanodine receptor antibodies were detected in 53.9%, 20.2%, 64.0% and 55.0% of myasthenia gravis patients respectively, higher than in patients of other neurological diseases and controls groups. The combination of the four antibodies assays provided 94.4% sensitivity and 84.0% specificity for the diagnosis of myasthenia gravis. Acetylcholinesterase antibody occurred more frequently in acetylcholine receptor antibody negative patients with adverse reactions to neostigmine test. Titin antibody provided 82.1% sensitivity and 52.5% specificity for myasthenia gravis with thymoma. Incidences of titin and of ryanodine receptor antibody were higher in late onset myasthenia gravis than in early onset myasthenia gravis. The proportion of titin antibody positive patients increased with the severity of myasthenia gravis as graded by a modified Osserman scale. Conclusions Testing for acetylcholine receptor, acetylcholinesterase, titin and ryanodine receptor antibodies can offer a better diagnostic method for myasthenia gravis than each antibody test alone. Titin antibody combined with computed tomography was better for the diagnosis of thymoma. Titin antibody occurred most frequently in severe myasthenia gravis.     

糖皮质激素对重症肌无力患者白细胞介素-6和乙酰胆碱受体抗体的影响

目的探讨糖皮质激素(GC)对重症肌无力(MG)患者白细胞介素-6(IL-6)和乙酰胆碱受体抗体(AchRab)的影响.方法采用ELISA法检测32例MG患者在用GC治疗前、治疗3个月后和20例正常对照者的血清IL-6和AchRab水平.结果MG患者血清IL-6和AchRab水平均显著高于对照组(P＜0.01)GC治疗能明显降低其水平(P＜0.05或P＜0.01),且对不同性别、年龄、病程、临床类型和不同预后的MG患者均有作用(P＜0.05或P＜0.01),但在GC治疗前后,预后差的MG患者血清IL-6和AchRab水平始终高于预后好者(P＜0.05).血清IL-6水平在AchRab阳性MG患者高于AchRab阴性患者(P＜0.05),且与AchRab滴度呈正相关(f=0.710,P＜0.01).结论GC可通过抑制MG患者体内IL-6和AchRab的产生,有效调节机体细胞免疫和体液免疫功能.     

抗乙酰胆碱受体单克隆抗体A7诱导实验性重症肌无力

目的:探讨抗乙酰胆碱受体单克隆抗体A7(Anti-AChR mAbA7)对实验性自身免疫性重症肌无力(EAMG)的作用.方法:Lewis大鼠腹腔注射5ml 20倍浓缩的含Anti-AChR mAbA7培养上清液,建立EAMG被动转移模型.对照组腹腔注射同体积的PBS.每8 h监测体重和临床症状评分.48h后处死实验动物.用放射免疫法检测AChR,并计算出AChR的损失率.结果:Anti-AChR mAbA7诱导的EAMG模型AChR损失率高,为24.3%～45.2%.而且AChR损失率与临床症状评分相关(r=0.74,P＜0.01).Anti-AChR mAbA7攻击的位点在终板的AChR上.结论:Anti-AChR mAb A7可用作诱导EAMG模型而应用于重症肌无力的研究.     

Predictors of postoperative myasthenic crisis in patients with myasthenia gravis after thymectomy

Background  Thymectomy is considered the most effective treatment in patients with myasthenia gravis. This study aimed to explore the predictors of postoperative myasthenic crisis in patients with myasthenia gravis after thymectomy.

Methods  Clinical records of 243 patients with myasthenia gravis who underwent thymectomy were reviewed retrospectively. The following factors were analyzed in relation to the occurrence of myasthenic crisis after thymectomy: gender, age, duration of symptoms, Osserman stage, history of myasthenic crisis, concomitant diseases, preoperative pyridostigmine dose, preoperative steroid therapy, operation approach, operation time, presence of thymoma, major postoperative complications.

Results  Forty-four patients experienced postoperative myasthenic crisis during the first month after thymectomy. Univariate analysis revealed that Osserman stage (RR=0.0976, P=0.000), history of myasthenic crisis (RR=0.2309, P=0.012), preoperative pyridostigmine dose (RR=0.4349, P=0.016), thymoma (RR=0.0606, P=0.000), and major postoperative complications (RR=0.1094, P=0.000) were significantly related to postoperative myasthenic crisis. Multivariate Logistic regression analysis showed that Osserman stage (IIb+III+IV) (RR=0.0953, P=0.000), thymoma (RR=0.0294, P=0.000), and major postoperative complications (RR=0.0424, P=0.000) independently predict postoperative myasthenic crisis.

Conclusion  Osserman stage (IIb+IIIb+IV), thymoma and major postoperative complications are independent predictors of postoperative myasthenic crisis in patients with myasthenia gravis who underwent thymectomy.

     

重症肌无力伴胸腺瘤患者术后放射治疗远期效果观察

目的:研究伴胸腺瘤的重症肌无力(MG)患者术后放射治疗的效果.方法:观察和比较60例伴胸腺瘤MG患者术后放疗与43例术后未放疗患者的远期疗效和生存率.结果:术后放疗组的50例(83.3％)患者放疗后MG症状好转.随访1～15年,MG总有效率为78.3％,其中完全缓解者12例(20.0％)、药物缓解者16例(26.7％);术后未放疗组MG总有效率为79.1％,其中完全缓解者16例(37.2％)、药物缓解者5例(11.6％),两组远期疗效差别无统计学意义(P>0.05).术后放疗组2、5和10年生存率分别为93.3％、80.0％和46.7％,其中Masaoka分期Ⅰ-Ⅱ期者分别为97.6％、92.8％和83.3％,Ⅲ-Ⅳ期者分别为83.3％、50.0％和0％;术后未放疗组2年、5年和10年生存率分别为86.0％、74.4％和48.8％,其中Ⅰ-Ⅱ期者分别为94.7％、86.8％和71.1％,Ⅲ-Ⅳ期者分别为40.0％、0％和0％.两组Ⅲ-Ⅳ期患者生存率比较具有统计学意义(P<0.01).结论:放射治疗可以提高伴浸润性胸腺瘤的MG患者远期生存率和缓解MG症状.     

重症肌无力的外科治疗

目的探讨重症肌无力的手术治疗及其效果。方法19倒重症肌无力病人，采用胸骨正中劈开切口（18例）和颈横切口（1例），彻底切除胸腺及纵隔内脂肪组织，术中不用肌松剂。对病情严重者胸腺切除同时或术后早期行气管切开。结果术后早期死亡1例，18例随访1mo～25a，症状完全缓解8例，改善8例，无变化1例，死亡1例。病理诊断：胸腺癌9例（其中恶性3例）；胸腺增生7例；胸腺结核1例；胸腺未见异常2例。结论无论病人年龄大小或胸腺病理类型如何，胸腺切除术治疗重症肌无力疗效满意。