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1.
采用ELISA法检测了202例重症肌无力(MG)患者血清肿瘤坏死因子(TNF)含量并与乙酰胆碱受体抗体(AChRab)含量进行同步对比研究结果发现,TNF和AChRab均值都明显高于正常对照组(P<0.01),109例患者口服强的松治疗后,其均值都较治疗前显著降低(P<0.01)。据此认为,TNF与MG发病有明显相关性,为MG的发病机理研究提供了实验室依据,并为观察MG病情轻重和评价免疫疗法治疗效果提供了实验室辅助指标。  相似文献   

2.
目的探讨乙酰胆碱受体抗体(AChR-Ab)与重症肌无力(MG)临床特征的相关性。方法采用放射免疫法检测115例MG患者及92例对照组(非MG神经系统疾病患者42例,健康体检者50名)血清AChRAb浓度,应用临床绝对评分记录MG患者病情严重程度。分析各组血清AChR-Ab浓度的差异,以及AChR-Ab浓度与MG患者临床特征的相关性。采用ROC工作特征曲线探讨AChR-Ab诊断MG的敏感度和特异度。结果MG患者血清AChR-Ab浓度中位数(四分位数间距,下同)为3.45(39.38)nmol/L,较非MG神经系统疾病患者[0(0)nmol/L]和健康体检者[0(0)nmol/L]增高(P0.01)。全身型MG(GMG)患者AChR-Ab浓度[25.45(46.14)nmol/L]较眼肌型MG(OMG)患者[0.58(3.56)nmol/L]增高(P0.01)。用ROC曲线法分析显示,以血清AChR-Ab浓度≥0.50nmol/L作为诊断MG界值时灵敏度为72.17%,特异度为100%,曲线下面积(AUC)=0.895(95%CI:0.849~0.941)。AChR-Ab浓度与发病年龄、病程及改良Osserman分型呈正相关(r=0.220,P0.05;r=0.184,P0.05;r=0.382,P0.01),但相关性较弱(均r0.5),与临床绝对记分无相关性(r=0.147,P0.05)。结论用放射免疫法检测血清AChR-Ab浓度诊断MG的灵敏度和特异度均高,有助于减少MG的漏诊率及误诊率,值得临床推广。  相似文献   

3.
目的比较非胸腺瘤性眼外肌麻痹起病重症肌无力(ocular onset myasthenia gravis,OMG-O)患者于不同病程行胸腺切除后病情缓解与向全身型重症肌无力(GMG)转化的差异,以期对手术时机进行探讨。方法采用双向队列研究方法,对作者医院2005年1月至2017年9月因药物治疗效果不佳而行胸腺切除的86例OMG-O患者,按起病至手术时间间隔分为早手术组(≤1年)、晚手术组(>1年),比较两组患者不同随访时间理想状态(干预后状态达到轻微异常及更好)率、GMG转化率的差异。结果 (1)与早手术组比较,晚手术组少年MG(<18岁)比例较高,起病至手术时间间隔、起病至使用激素时间间隔、激素用药时间较长(均P <0.05)。(2)与晚手术组比较,早手术组术后第3年理想状态率高(P=0.029),而第2、4~8年理想状态率有升高趋势,但差异无统计学意义(P>0.05)。(3)9例术后发生GMG转化,起病至发生GMG转化时间中位数17.8个月,其中6例(66.7%)在3年内发生GMG转化。早手术组与晚手术组间GMG转化率比较,差异无统计学意义(12.5%比8.7%;χ~2=0.049,P=0.825)。结论对于药物治疗无效或不耐受的OMG-O患者,早期行胸腺切除手术疗效较好;但即使手术,仍有可能发生GMG转化,约1/2患者于起病后1.5年内发生,约2/3于起病后3年内发生。  相似文献   

4.
目的探讨自身免疫性调节因子(AIRE)、滤泡辅助性T(Tfh)细胞和滤泡调节性T(Tfr)细胞与重症肌无力(MG)患者病情严重程度的相关性。方法收集2015-12—2016-4第四军医大学唐都医院收治的MG患者22例,根据临床表现分为全身型MG(GMG)和眼肌型MG(OMG);同期选取健康体检中心查体者10名作为健康对照。收集MG患者详细临床资料,包括美国MG协会(MGFA)分型及定量MG(QMG)评分。通过流式细胞术分析AIRE阳性细胞比例及Tfh/Tfr比值。结果 (1)AIRE表达在各组间比较差异具有统计学意义(P0.01)。GMG组和OMG组AIRE表达均较对照组降低(P0.01,P0.05),而GMG组与OMG组间比较差异无统计学意义(P0.05)。(2)Tfh/Tfr比值在各组间比较差异具有统计学意义(P0.01)。GMG组和OMG组Tfh/Tfr比值均高于对照组(P0.01,P0.05),且GMG组高于OMG组(P0.05)。(3)MG患者AIRE表达与MGFA分型及QMG评分呈负相关(r=-0.517,P0.05;r=-0.616,P0.01),Tfh/Tfr比值与MGFA分型和QMG评分呈正相关(r=0.761,r=0.581,均P0.01)。结论 AIRE、Tfh/Tfr比值与MG的病情严重程度有一定的相关性,并可能参与了MG的发病。  相似文献   

5.
目的 :探讨血清阳性 (SPMG)和阴性重症肌无力 (SNMG)被动转移动物模型 (P EAMG)的异同。方法 :用ELISA法将重症肌无力 (MG)患者分为SNMG和SPMG两组 ,然后分别用两组患者血清制作P EAMG ,观察两组小鼠的临床表现、电生理及神经肌接头(NMJ)的改变。结果 :SPMG和SNMG组小鼠均表现出明显的肌无力症状 ,低频重复电刺激出现明显衰减反应 ,但SNMG组小鼠肌无力症状较SPMG组明显为轻 ,SPMG和SNMG组小鼠NMJ处棕黄色沉积物明显减少、变细短。结论 :SNMG和SPMG均是自身抗体介导的自身免疫性疾病 ,但两者不完全相同  相似文献   

6.
目的:研究CD5+B细胞在重症肌无力(MG)发病中的作用。方法:采用免疫荧光双标记技术和流式细胞仪对39例MG患者和18例健康对照者周围血中CD5+B细胞(CD5+CD19+)的百分率进行测定,同时用ELISA间接法检测MG患者血清中AChRab。结果:在MG患者周围血CD5+B细胞百分率显著高于对照组,而且在血清AChRab阳性和阴性MG中CD5+B细胞均明显高于对照组;但在血清AChRab阳性和阴性MG患者之间CD5+B细胞百分率无显著性差异发现。结论:CD5+B细胞与MG发病有关,但与AChRab的产生之间无显著相关。  相似文献   

7.
本研究旨在探讨长期以来无定论的重症肌无力(MG)患者血和脑脊液(CSF)中的乙酰胆碱受体抗体(AChRab)能否与中枢神经元烟碱型乙酰胆碱受体(神经-nAChR)结合,并引起中枢神经系统(CNS)功能障碍。用免疫亲和层析法从AChRab阳性的全身型MG患者血中提取纯化AChRab,然后用免疫组化法探讨AChRab与大鼠中枢神经-nAChR之间的免疫结合反应。结果首次表明,AChRab与神经-nAChR之间的阳性免疫结合反应广泛分布于大鼠大脑皮层、脑干颅神经运动核团、脊髓前角运动神经元等部位,提示MG患者AChRab不仅可与神经肌接头(NMJ)处肌-nAChR结合引起肌无力等症状,还可与CNS神经-nAChR结合,并可能引起CNS功能障碍。  相似文献   

8.
Myasthenia gravis (MG) is a kind of chronic autoimmune disease which can weaken patients' motor function and, furthermore, produce negative impact on the health-related quality of life (HRQoL). The primary purpose of this research was to evaluate factors that might affect the HRQoL of MG patients. A cross-sectional clinical research was carried out including 188 successive patients with MG. Myasthenia Gravis Foundation of America (MGFA) classification and Quantitative Myasthenia Gravis (QMG) score were applied to assess the severity of the disease. The Medical Outcome Survey 36-Item Short-Form Health Survey (SF-36) was used to estimate the HRQoL. Hamilton Depression Rating Scale (HDRS) and Hamilton Anxiety Rating Scale (HARS) were utilized to measure the depression and anxiety symptom. Factors may influence the HRQoL of MG patients include age, educational level, occupation, the situation of the thymus, the type of MG and generalized myasthenia gravis (GMG), the severity of the disease and the psychological disorder. Higher QMG and HARS scores were two significant factors that can prognosticate lower Physical Composite Score (PCS) and Mental Composite Score (MCS), while older age was just a significant factor which has prognostic value for lower PCS. The results of this research may have a potential guiding significance for the clinical treatment strategy and improve the quality of life in patients with MG consequently. In addition to the treatment of physical symptoms, the psychological symptoms such as anxiety and depression should be concerned as well.  相似文献   

9.
目的分析青少年起病的重症肌无力(MG)患者的临床特点。方法回顾性分析2006-2013年作者医院神经内科住院的351例起病年龄≤18岁MG患者的临床资料,包括人口统计学(性别、年龄),临床特点(临床分型、起病症状、疾病转归、胸腺情况)和合并疾病(自身免疫性疾病)。结果青少年起病的MG患者发病年龄小于5岁者占45.9%,女性多见占53.6%。根据美国重症肌无力协会(myasthenia gravis foundation of America,MGFA)分型,Ⅰ型占80.3%,Ⅱa型占8.5%,Ⅱb型占9.1%,Ⅲa型占0.3%,Ⅲb型占1.4%,Ⅳb型占0.3%。由眼肌型MG(OMG)转化为全身型MG(GMG)患者39例(13.8%),主要诱发转化的因素为不规律用药或停药。诱发病情复发或加重的因素中以受凉多见,占63.6%。合并胸腺异常患者有60例,其中28例行胸腺切除术,术后病理分型:胸腺增生20例,胸腺瘤4例。合并有其他自身免疫性疾病共20例,其中甲状腺功能亢进占4.6%,甲状腺功能减低占0.6%,甲状腺囊肿占0.6%。共有6例患者发生过危象,其中5例发生肌无力危象者为由OMG转化为GMG患者。结论青少年MG患者有自己独特的临床特点:5岁之前发病更多见;诱发病情复发或加重的因素中以受凉多见;合并胸腺增生者多于合并胸腺瘤者;最常见的甲状腺功能异常为甲状腺功能亢进。  相似文献   

10.
目的探讨血清抗乙酰胆碱受体抗体(AChRab)、抗突触前膜抗体(PsMab)和柠檬酸提取物相关抗体(CAEab)在胸腺异常患者中的诊断价值。方法回顾性收集2009—2016年作者医院收治的经病理检查证实存在胸腺异常的患者152例,同时收集健康体检者45名(NC组)和其他神经系统疾病患者35例(OND组)作为对照组。采用ELISA方法检测各组AChRab、PsMab和CAEab水平,比较各组不同抗体阳性率的差异,并进一步比较不同胸腺异常者间各抗体阳性率差异。结果 (1)胸腺瘤、胸腺增生组血清AChRab、PsMab和CAEab阳性率明显高于NC及OND组(均P0.01),且胸腺瘤组上述3种抗体阳性率高于胸腺囊肿组(P0.01或P0.05)。胸腺囊肿组与NC及OND组各抗体阳性率比较无统计学差异(P0.05)。CAEab对胸腺瘤患者诊断灵敏度为60.6%,特异度为88.8%。(2)重症肌无力合并胸腺瘤(MGT)组PsMab阳性率高于非合并MG胸腺瘤(NMGT)组(P0.05),而其AChRab及CAEab阳性率与NMGT组比较无统计学差异(P0.05)。(3)MGT组中,MGT-全身型组血清AChRab阳性率较MGT-眼肌型组增高(P0.05)。(4)胸腺瘤不同病理分型患者间各抗体阳性率比较无统计学差异(均P0.05)。结论 (1)CAEab检测结合CT检查可作为临床可疑胸腺瘤、胸腺增生患者术前辅助诊断指标之一。(2)AChRab阳性可能与MGT患者重症肌无力严重程度有关,PsMab阳性可能跟MGT患者致病有关。  相似文献   

11.
Objectives –  To examine myesthenia gravis (MG) severity and long-term prognosis in seronegative, seropositive, and thymoma MG.
Materials and Methods –  Four series of patients were studied retrospectively. Severity and treatment were assessed each year, and muscle antibodies were assayed.
Results –  Seropositive MG patients had a more severe course than seronegative MG patients. MG severity was higher in non-thymectomized compared to thymectomized early-onset MG patients. MG severity did not differ between thymectomized and non-thymectomized late-onset patients. There was no significant difference in MG severity between thymoma and non-thymoma MG patients.
Conclusions –  MG is more severe in seropositive MG patients. With proper treatment, especially early thymectomy, the long-term prognosis is good in seropositive MG patients. The present studies indicate a benefit of thymectomy in early-onset MG, but no dramatic benefit in late-onset MG. Similar MG severity and outcome was seen in thymoma and non-thymoma MG.  相似文献   

12.
Objective: The clinical differences of early-onset myasthenia gravis (EOMG) and late-onset MG (LOMG) have not been elucidated in China. In order to clarify this, a retrospective study was conducted in 985 MG patients, whose disease duration was longer than 3 years.

Methods: These patients were separated into EOMG and LOMG according to the onset age of 50 years. The clinical differences including demographics, clinical features, thymus abnormalities and comorbidities of EOMG and LOMG patients were analyzed.

Results: Results indicated that 485 were males and 500 were females, 714 were EOMG and 271 were LOMG. Female was more common in EOMG and male was more common in LOMG (p = 0.003). The peak onset age was 0–4 years in EOMG and 55–59 years in LOMG. Ocular MG (OMG) was more common in EOMG and generalized MG (GMG) was more common in LOMG (p = 0.004). The transformation rate of OMG to GMG was higher in LOMG (p = 0.002). The positive incidence of repetitive nerve stimulation (RNS) was higher in EOMG (p = 0.026). Thymoma was more frequent in LOMG (p = 0.017) and thymic hyperplasia was more frequent in EOMG (p < 0.001). Hyperthyroidism was more common in EOMG (p = 0.017) and diabetes was more common in LOMG (p < 0.001).

Conclusion: These results have potential significance for the recognition of clinical features and the determination of management strategies in EOMG and LOMG.  相似文献   


13.
目的探讨重症肌无力(myasthenia gravis,MG)患者汉密尔顿抑郁量表(Hamilton depression rating scale,HDRS)评分情况及其影响因素分析。方法横断面研究2013-07—2015-03作者医院就诊的188例MG患者的临床资料和HDRS评分情况,并根据HDRS评分将其分为抑郁组和非抑郁组,分析两组MG患者的临床特点及其与HDRS评分间的关系。结果所纳入MG患者男女比例为1.02∶1,眼肌型重症肌无力(ocular myasthenia gravis,OMG)和全身型重症肌无力(generalized myasthenia gravis,GMG)的比例为1.2∶1,以OMG起病和以GMG起病患者的比例为6.2∶1,病程中位数为2年,四分位数间距为1.8年,平均量化重症肌无力评分(quantitative myasthenia gravis,QMG)为(6.7±2.3)分,平均HDRS评分为(8.7±3.4)分,并发抑郁者65例,未并发抑郁者123例。影响HDRS评分和抑郁发生的相关因素包括性别(P0.01)、MG类型(P0.01)、QMG得分(P0.01)和美国重症肌无力协会(myasthenia gravis foundation of America,MGFA)分型(P0.01)、有无甲状腺功能亢进(P0.05)。结论影响MG患者HDRS评分和抑郁发生的相关因素包括性别、MG类型、QMG评分和MGFA分型、有无甲状腺功能亢进,充分认识其抑郁发生情况有利于更好地治疗MG。  相似文献   

14.
The complement C3b/C4b receptor (CR1) expression on erythrocytes (E) (ECR1) from patients with myasthenia gravis (MG) and from blood donors was measured using haemadsorption and indirect hemagglutination techniques. The ECR1 expression in patients with MG appeared to be normal. This was also true for the subgroup of MG patients with additional autoimmune diseases. No correlation between ECR1 expression and severity of myasthenic symptoms was demonstrated.  相似文献   

15.
Objective To evaluate the frequency, distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor (AChR) in patients with myasthenia gravis (MG). Methods AChR antibodies were detected by cell-based assay in the serum of ocular MG (OMG) (n = 90) and generalized MG (GMG) patients (n = 110). The fetal-type (2α: β: γ: δ) and adult-type (2α: β: ε: δ) AChR were used as antigens, and their relevance to disease presentation was assessed. Results The overall frequencies of anti-adult and anti-fetal AChR antibodies were similar in all 200 patients examined, with 14 having serum specific to the AChR-γ subunit, and 22 to the AChR-ε subunit. The overall sensitivity when using the fetal and adult AChR antibodies was higher than that when using the fetal AChR antibody only (P = 0.015). Compared with OMG patients, the mean age at disease onset and the positive ratio of antibodies to both isoforms of the AChR were significantly higher in patients who subsequently progressed to GMG. Older patients and patients with both anti-fetal and anti-adult AChR antibodies had a greater risk for developing generalized disease [odds ratio (OR), 1.03; 95% confidence interval (CI), 1.01-1.06 and OR, 5.09; 95% CI, 2.23-11.62]. Conclusion Using both fetal-and adult-type AChRs as the antigens may be more sensitive than using either subtype. Patients with serum specific to both isoforms are at a greater risk of progressing to GMG. Patients with disease onset at an advanced age appear to have a higher frequency of GMG conversion.  相似文献   

16.
抗体阴性重症肌无力发病与凝集素之间关系研究   总被引:2,自引:0,他引:2  
借助研究抗体阴性重症肌无力(MG)发病与凝集素之间的关系,以阐明其发病过程是否与凝集素有关。方法观察伴刀豆球蛋白A(ConA)和麦胚凝集素(Triticum)及其凝集素-糖复合物对TE671细胞表达的乙酰胆碱受体(AChR)功能的作用,以及对α-BuTx结合试验的影响。结果有两种凝集素对AChR功能均有抑制作用,抑制率(%)分别为54±14(n=11)和47±16(n=10),此作用可被3种糖抑制,抑制率(%)分别为:95±5(n=5)和84±8(n=5);69±6(n=4)和65±5(n=4);39±4(n=5)和57±6(n=5);ConA抑制α-BuTx结合试验,而Triticum则不能。结论Triticum和抗体阴性MG患者非IgG部分对AChR功能和α-BuTx结合试验的作用类同或一致,表明抗体阴性MG患者非IgG部分中的内源性Triticum样糖蛋白在其发病过程中起重要作用。  相似文献   

17.
目的研究重症肌无力(myasthenia gravis,MG)患者外周血辅助性T细胞22(T helper 22cells,Th22)和白细胞介素-22(interleukin-22,IL-22)的表达以及两者间的相关性。方法收集25例MG患者和24例健康对照者,其中眼肌型重症肌无力(ocular myasthenia gravis,OMG)患者14例,全身型重症肌无力(general myasthenia gravis,GMG)患者11例。采用流式细胞仪检测MG患者和健康对照者外周血单个核细胞(peripheral blood mononuclear cells,PBMC)中Th22细胞的比例,采用酶联免疫吸附实验(enzyme-linked immunosorbent assay,ELISA)检测血浆IL-22的表达。比较各组间Th22细胞比例和IL-22表达水平差异,以及Th22细胞比例和IL-22表达间的相关性。结果 MG患者PBMC中Th22细胞比例、血浆IL-22表达水平均显著低于健康对照组[(0.60±0.07)%比(0.92±0.09)%,P0.01;(18.65±1.38)pg/mL比(24.54±1.85)pg/mL,P0.05];OMG与GMG患者间Th22细胞比例、IL-22表达水平均无统计学差异(均P0.05);MG患者PBMC中Th22细胞比例与IL-22表达水平间呈中度正相关(r=0.59,P0.01)。结论 MG患者体内Th22细胞比例及血浆IL-22表达水平减低可能导致免疫功能紊乱进而影响MG的发病。  相似文献   

18.
We investigated the effect of the lgG from patients with myasthenia gravis (MG) on the degradation of normal rat junctional acetylcholine receptor (AChR) labeled with 125l-α-bungarotoxin (BuTx) and calculated the degradation rate (DR). The DR for the lgG from these patients was significantly higher than that from healthy volunteers and patients with other autoimmune diseases. For MG, DR was significantly correlated with the severity of the disease but not with anti-AChR antibody titer. DR was accelerated by lgG from patients with generalized MG whose antibody titers were in the normal range and by lgG from patients with ocular MG. These results indicate that measurement of the DR of junctional AChR in normal rats is more closely correlated with the severity of the disease than is measurement of anti-AChR antibody and that the former is a sensitive and confirmatory method for evaluating MG. © 1993 John Wiley & Sons, Inc.  相似文献   

19.
Asymptomatic myasthenia gravis influences pregnancy and birth   总被引:1,自引:0,他引:1  
Women with myasthenia gravis (MG) have an increased risk of complications and adverse pregnancy outcome. This study has examined if this is true also for asymptomatic MG. Using data from the Medical Birth Registry of Norway, births of women prior to receiving an MG diagnosis or in complete clinical MG remission were compared with all non-MG births in Norway in the same period (1967-2000). Forty-nine births occurred in 37 women, 11 of them in clinical remission, and six thymectomized. The perinatal mortality was increased (P = 0.02) and induction of birth (P = 0.007) occurred more frequently. Protracted labor occurred more frequently in the target group (P = 0.03). One of the three children that died had Potter's syndrome. Both mothers with children who died were in complete clinical MG remission. One had previously given and one subsequently gave birth to a child with neonatal MG. The results indicate that complications in birth and pregnancy are not only related to clinical MG disease severity but to the underlying immunological dysfunction.  相似文献   

20.
目的探讨重症肌无力(MG)患者外周血中Th17细胞及相关细胞因子白细胞介素17(IL-17)在MG发病中的作用。方法收集40例MG患者和10名健康人(对照组)外周血标本,采用流式细胞术检测外周血单个核细胞(PBMCs)中Th17细胞比例,反转录酶-聚合酶链锁反应(RT-PCR)检测PBMCs中维甲酸受体相关孤儿受体γt(RORγt)mRNA水平,ELISA检测血清中IL-17水平,放射免疫沉淀法检测血清中抗乙酰胆碱受体抗体(AChR-Ab)滴度;分离PBMCs中CD4~+T细胞和CD19~+B细胞与金黄色葡萄球菌肠毒素B(SEB)进行共培养,培养系统中加入人IL-17和(或)IL-21中和抗体,放射免疫测定法检测培养液中AChR-Ab滴度。采用MG评分(quantitative MG scoring system,QMGs)对MG的严重程度进行评估,并对MG患者的Th17细胞比例、RORγt mRNA和IL-17水平与病情QMGs的相关性,以及MG患者抗AChR-Ab滴度与PBMCs中Th17细胞比例的相关性进行分析。结果 MG患者PBMCs中Th17细胞比例[1.11%(0.90%,1.34%)]高于健康对照组Th17细胞比例[0.26%(0.08%,0.36%)](z=5.494,P0.001),且与疾病严重程度呈正相关(r=0.4394,P=0.0046);血清中IL-17水平和PBMCs中RORγt mRNA相对表达[分别71.46(53.91,104.76)pg/mL、2.63(1.94,3.12)]均较健康对照组[分别18.82(12.73,29.80)pg/mL、1.13(0.98,1.28)]显著增高(均P0.001);MG患者血清中抗AChR-Ab滴度[2.34(1.19,3.60)nmol/L]较健康对照组[-0.08(-0.24,-0.03)nmol/L]显著增高(z=4.662,P0.001),且与Th17细胞比例呈正相关(r=0.7066,P=0.0001)。MG患者外周血T、B细胞与SEB共培养后抗AChR-Ab水平高于未加入SEB时及健康对照(均P0.01);加入抗人IL-21或IL-17中和抗体后,两者AChR-Ab滴度与未加入抗体时AChR-Ab滴度比较均降低(均P0.05),且均仍高于MG患者未加入SEB时及健康对照(P0.01);在培养上清中同时加入抗人IL-21和IL-17中和抗体时AChR-Ab滴度明显低于加入单种抗体时,而与未加入SEB时及健康对照差异无统计学意义(均P0.05)。结论 MG患者外周血中Th17细胞可能通过IL-17促进AChR-Ab产生,参与疾病的病理过程。  相似文献   

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