眼肌型重症肌无力治疗措施的临床证据

目的为眼肌型重症肌无力患者循证制定最佳治疗方案。方法检索PubMed（1966～2010.4）、Cochrane图书馆（2010年第1期）、中国知识资源总库（1979～2010.4）及中文科技期刊数据库（1989～2010.4）,获取并评价有关胆碱酯酶抑制剂、糖皮质激素、免疫球蛋白、免疫抑制剂、血浆置换和胸腺切除的临床指南、系统评价、随机对照试验、临床对照试验及病例观察研究。结果共纳入与眼肌型重症肌无力治疗有关的治疗指南2篇、系统评价5篇、观察性研究4篇,其分别评价了胆碱酯酶抑制剂、糖皮质激素、免疫球蛋白、免疫抑制剂及胸腺切除的疗效与安全性。眼肌型重症肌无力患者应首选溴吡斯的明治疗,若患者不能自发缓解,应在充分评估使用糖皮质激素的利弊后考虑使用激素治疗,激素疗效欠佳时可考虑加用免疫抑制剂长期服用以缓解症状。血浆置换术不作为眼肌型重症肌无力的推荐治疗,对合并胸腺瘤的患者应施行胸腺切除术。结论借助循证医学的方法可为眼肌型重症肌无力患者的治疗提供最佳临床证据。     

重症肌无力的多样性表现和治疗

重症肌无力是一种由神经肌肉接头处的肌肉乙酰胆碱受体(AChR)抗体所致的自身免疫性疾病。神经肌肉接头处的乙酰胆碱受体缺损导致神经肌肉传导不良,从而引起肌肉无力及疲劳。近年来重症肌无力的发病率有所上升,特别是在老年人,这可能与AChR抗体检测技术的广泛应用使得重症肌无力能得到及时准确的诊断有关。重症肌无力在发病年龄、是否伴有胸腺改变以及累及肌肉的分布等方面表现多样,但自身免疫因素及胸腺异常在不同类型重症肌无力发病中的作用仍不清楚。目前大多数患者应用胆碱酯酶抑制剂及免疫抑制剂获得有效治疗,对于年轻患者则施行胸腺切除。大约有15%的重症肌无力患者AChR抗体阴性,这类患者大多存在另一个神经肌肉接头蛋白抗体,即肌肉特异性激酶(MuSK)抗体。     

重症肌无力的多样性表现和治疗

重症肌无力是一种由神经肌肉接头处的肌肉乙酰胆碱受体(AChR)抗体所致的自身免疫性疾病。神经肌肉接头处的乙酰胆碱受体缺损导致神经肌肉传导不良，从而引起肌肉无力及疲劳。近年来重症肌无力的发病率有所上升，特别是在老年人，这可能与AChR抗体检测技术的广泛应用使得重症肌无力能得到及时准确的诊断有关。重症肌无力在发病年龄、是否伴有胸腺改变以及累及肌肉的分布等方面表现多样，但自身免疫因素及胸腺异常在不同类型重症肌无力发病中的作用仍不清楚。目前大多数患者应用胆碱酯酶抑制剂及免疫抑制剂获得有效治疗，对于年轻患者则施行胸腺切除。大约有15％的重症肌无力患者AChR抗体阴性，这类患者大多存在另一个神经肌肉接头蛋白抗体，即肌肉特异性激酶(MuSK)抗体。     

干涸疗法治疗术后重症肌无力危象的护理探讨

目的：总结干涸疗法治疗重症肌无力术后并发重症肌无力危象的护理经验。方法：对23例重症肌无力术后并发重症肌无力危象的病人实行干涸疗法，针对其长期应用呼吸机，心理负担重、机体抵抗力低、体能消耗大等特点，实施有重点的身心护理。结果：21例病人安全度过危险期，2例病人难以撤离呼吸机，行血浆置换后脱离危象。结论：干涸疗法是一种治疗手术后重症肌无力危象的有效方法，加强呼吸机使用及停用过程的护理，使用免疫抑制剂副作用的观察和预防，加强心理护理和营养支持，是护理干涸疗法治疗手术后重症肌无力危象病人的关键。     

外科治疗重症肌无力50例围术期的护理

重症肌无力是胸腺疾病引发的自身免疫性疾病。手术切除胸腺是治疗重症肌无力有效和首选方法.1998-01／2008-12我科收治重症肌无力50例，均采用了外科治疗，现就护理情况分析如下。     

重症肌无力胸腺切除的麻醉处理

重症肌无力是神经肌肉接头处传导功能障碍,同胸腺有关的自身免疫性疾病.除药物、免疫、激素、放疗外,现多主张重症肌无力都适于胸腺切除。自1982年来我院对7例重症肌无力病人行胸腺切除,有关麻醉处理报告如下。     

短程大剂量糖皮质激素冲击联合其他免疫抑制剂治疗重症肌无力的疗效观察

目的探讨短程大剂量糖皮质激素冲击联合其他免疫抑制剂对重症肌无力的临床疗效。方法选取80例重症肌无力患者,对照组患者给予糖皮质激素,实验组患者则采用短程大剂量糖皮质激素冲击联合免疫抑制剂,比较2组患者定量重症肌无力症状评分、总体治疗效果、生存质量与不良反应。结果实验组患者治疗后各维度定量重症肌无力症状评分与显效率均显著高于对照组,各维度生存质量评分与总体不良反应率均显著低于对照组(P0.01)。结论短程大剂量糖皮质激素冲击联合其他免疫抑制剂对重症肌无力的临床疗效显著。     

胸腺切除治疗重症肌无力的术后护理对策

重症肌无力(MG)是一种自身免疫性疾病,胸腺切除是治疗MG的重要方法,中国人民解放军总医院胸外科1989年～2003年对86例重症肌无力患者进行胸腺切除手术,未发生手术及住院死亡,全部患者均痊愈出院,现总结分析护理问题与应对策略,以此提高胸腺切除治疗重症肌无力的术后护理水平.     

胸腺切除治疗重症肌无力的术后护理对策

重症肌无力（MG）是一种自身免疫性疾病，胸腺切除是治疗MG的重要方法，中国人民解放军总医院胸外科1989年～2003年对86例重症肌无力患者进行胸腺切除手术，未发生手术及住院死亡，全部患者均痊愈出院，现总结分析护理问题与应对策略，以此提高胸腺切除治疗重症肌无力的术后护理水平。     

胸腺切除治疗儿童重症肌无力

儿童重症肌无力是儿童期较少见的疾病之一,为一自身免疫性疾病,其发病与神经肌肉联结的传递障碍有关.胸腺切除治疗儿童重症肌无力是目前公认的有效治疗方法,我院从1988～1998年对20例儿童重症肌无力患者施行胸腺切除术,14例患者获得随访,现将结果报告如下.     

Striational autoantibodies: quantitative detection by enzyme immunoassay in myasthenia gravis, thymoma, and recipients of D-penicillamine or allogeneic bone marrow

Striational autoantibodies (StrAb) are a useful serologic marker of thymoma in patients with myasthenia gravis (MG). We compared a standard immunofluorescence method with a new enzyme immunoassay (EIA) for detection of StrAb. Retrospective testing of 264 stored sera by the two methods yielded well-correlated results (58 sera were positive by both assays; r = 0.8). For 104 patients with spontaneously acquired MG or thymoma, results were 100% concordant, of which 53% were positive. For 34 recipients of D-penicillamine, StrAb were found in 15% by EIA and in 6% by immunofluorescence. StrAb were detected in two of four bone marrow recipients by EIA and in one by immunofluorescence. Prospective testing of 434 fresh sera (of which 49 were positive by the two methods) yielded discordant results in only 4. Serial EIA quantitation of StrAb in two patients with MG and thymoma proved useful in monitoring immunosuppressant therapy and in a third patient predicted recurrence of the tumor. A high prevalence of StrAb was detected by both assays in elderly patients with spontaneous MG, but StrAb were more readily quantifiable by EIA. The EIA method proved to be highly sensitive and specific for detecting StrAb in patients with thymoma with and without MG, in patients treated with D-penicillamine, and in those with graft-versus-host disease after bone marrow transplantation.     

Therapeutic algorithm

Therapeutic algorism for myasthenia gravis (MG) and myasthenic syndrome are described. For adult MG patients, thymectomy is basically recommended as an essential therapy. However, drug therapy or surgical treatment may be chosen for child MG patients depending on their age of onset. As for Lambert-Eaton myasthenic syndrome (LEMS), lung cancer and other malignancy may be treated first especially in male patients, and then drug therapy may be considered.     

伴胸腺瘤的重症肌无力患者治疗和预后

目的研究伴有胸腺瘤的重症肌无力(MG)患者的治疗方法和预后.方法回顾性研究20例经手术证实的MG伴胸腺瘤患者.给予皮质类固醇、放射疗法等治疗,观察患者的远期疗效和生存率.结果显示远期有效率80%(16/20),其中3例(15%)获完全缓解、10例(50%)获药物缓解、3例(15%)症状明显改善;1例症状不变;5年存活率88.9%、10年存活率57.1%;结论手术治疗MG伴胸腺瘤患者远期疗效良好,术后应给予皮质类固醇和放疗等综合治疗,以提高疗效和患者生存率.     

胸腺切除对重症肌无力患者外周血淋巴细胞Fas表达及T淋巴细胞亚群的影响

目的研究胸腺切除对重症肌无力（MG）患者外周血淋巴细胞（PBL）中Fas表达及T淋巴细胞亚群的影响,探讨胸腺切除术治疗MG与Fas介导的细胞凋亡的关系。方法采用流式细胞技术检测17例MG患者及13例健康对照组PBL中CD4^＋CD8^＋及Fas表达。结果与对照组相比,无行胸腺切除术组MG患者PBL中CD4^＋CD8^＋细胞比例升高,CD4^＋CD8^＋细胞比例下降,差异有统计学意义（P〈0．05）;行胸腺切除术组CD4^＋CD8^＋细胞比例较无行胸腺切除术组下降,差异有统计学意义（P〈0．05）。无行胸腺切除术组MG患者PBL中Fas^＋、CD4^＋Fas^＋细胞比例以及行胸腺切除术组Fas^＋细胞比例均高于对照组,差异有统计学意义（P〈0．05）。行胸腺切除术组MG患者PBL中Fas^＋、CD4^＋Fas^＋及CD4^＋Fas^＋细胞比例低于无行胸腺切除术组,但差异无统计学意义（P〉0．05）。结论MG患者存在外周血T淋巴细胞亚群分布异常,胸腺切除可减少此异常。MG患者PBL表面Fas表达升高,可能反映体内T淋巴细胞的活化及CD8^＋T淋巴细胞介导的细胞毒性作用增强。胸腺切除可降低PBL中Fas的表达,可能与治疗MG的机制有关。     

Comparative evaluation of the microcolumn gel card test and the conventional tube test for measurement of titres of immunoglobulin G antibodies to blood group A and blood group B

The conventional tube test (CTT) and microcolumn gel card test (MG) were used to measure immunoglobulin G (IgG) antibody titres to blood group A (anti-A) and blood group B (anti-B) in 288 serum samples. Diagnostic concordance and correlations between the two methods were analysed. MG was more sensitive than CTT in the detection of both anti-A and anti-B IgG. Correlation coefficients for the two methods for the titration of anti-A and anti-B were 0.8470 and 0.8336, respectively. The regression equation representing the relationship between the two methods was calculated and used to convert critical values for anti-A and anti-B IgG antibodies determined by CTT to critical values for MG titration. Analyses showed 86.8% and 89.9% diagnostic concordant rates for the detection of anti-A and anti-B IgG, respectively. It was concluded that titration results obtained by MG correlate well with those obtained by CTT, but critical values determined by CTT need to be converted into corresponding MG values.     

Sequential plasmapheresis and intravenous immunoglobulin therapy for refractory myasthenia gravis: case report.

Immunotherapy is currently the standard therapy for myasthenia gravis (MG) although some patients may be refractory to treatment. We describe the use of sequential plasmapheresis and intravenous immunoglobulin (IVIG) therapy for treatment of advanced MG in a patient refractory to all forms of medical treatment including corticosteroids, immunosuppressants, and intermittent plasmapheresis. The patient, a 37-year-old woman with systemic lupus erythematosus (SLE), had initially responded well to treatment with high dose corticosteroids and intermittent plasmapheresis, with the duration of response ranging from 3 to 4 months. However, after 18 months of therapy, the duration of response had gradually decreased to 1 month. She responded well to a 5 day trial of plasmapheresis followed by high dose IVIG, and the duration of response increased to 6 months. The SLE activity was relatively silent during each relapse. This report indicates the potential usefulness of sequential plasmapheresis and IVIG in the treatment of patients with refractory MG and SLE.     

Therapeutic apheresis in myasthenia gravis.

Plasma exchange (PE) is an easily applicable technique for rapid and massive removal of antibodies, and its beneficial role is well established in the management of myasthenia gravis (MG), an antibody-mediated disorder of the neuromuscular junction. PE is useful in myasthenic crisis, in most severe forms of MG before thymectomy, in the early postoperative period, and in cases of symptom worsening during tapering or initiation of immunosuppressive therapy. Clinical efficacy varies from 55% to 100%, and improvement rarely persists for more than 4-10 weeks; thus immunosuppressive therapy has to be associated. New apheretic techniques (double filtration plasmapheresis, immunoadsorption systems with staphylococcal protein A columns or thryptophan-polyvinyl alcohol gel columns) that allow the selective removal of IgG and anti-AChR antibody were recently used in the management of MG with positive effects. Whether their therapeutic effect and cost effect prove more favorable than those obtained by PE still must be demonstrated.     

视频镜像疗法对卒中后肩痛的疗效观察

目的:观察视频镜像疗法对卒中后肩痛的疗效。方法:将符合纳入标准的40例患者随机分为对照组和镜像组各20例,进行为期4周,每周5d的康复训练。对照组患者仅接受常规康复训练,镜像组在常规康复训练基础上每天增加30min镜像训练。在治疗前以及治疗后4周对2组患者进行数字分级量表(NRS)评估肩关节在主动和被动运动下的疼痛程度;Fugl-Meyer运动功能评分上肢部分(FMA-UE)进行上肢运动功能评定;功能独立性评分(FIM)运动部分评估日常生活独立能力。结果:治疗4周后,2组NRS评分均较治疗前显著降低(均P<0.01),FMA-UE及FIM评分均较治疗前显著提高(均P<0.01);治疗4后,镜像组的主、被动NRS评分显著低于对照组(P<0.05),而镜像组的FMA-UE及FIM评分显著高于对照组(P<0.05)。结论:视频镜像疗法可显著改善卒中患者的肩痛、上肢运动功能以及日常生活独立能力。     

Induction of myasthenia gravis,myositis, and insulin-dependent diabetes mellitus by high-dose interleukin-2 in a patient with renal cell cancer

Interleukin-2 is an effective agent against renal cell carcinoma and melanoma, but it has been associated with autoimmune sequelae such as hypothyroidism and vitiligo. A 64-year-old man with non-insulin-dependent diabetes and metastatic renal cell carcinoma developed insulin-dependent diabetes after his first cycle of therapy with high-dose (HD) interleukin-2. After additional therapy with interleukin-2, the patient developed generalized myasthenia gravis (MG) and polymyositis, both of which responded to treatment with corticosteroids and plasmapheresis. To investigate the role of IL-2 in the development of these autoimmune complications, autoantibody titers were assayed from serum obtained before and after IL-2 treatment and after treatment with corticosteroids plus plasmapheresis. Before IL-2 treatment, the patient had antibodies directed against insulin, islet cell antigens, and striated muscle. Acetylcholine receptor antibody levels were normal before starting IL-2. After treatment with IL-2, the patient developed acetylcholine receptor binding antibodies and exhibited an increase in the striated muscle antibody titer from 1:40 to 1:160. Recovery from the MG and polymyositis was associated with substantial decreases in the acetylcholine receptor and striated muscle antibody titers. These findings suggest that HD IL-2 accelerated the progression of latent autoimmune diabetes and myositis in this patient whose tolerance to islet cell antigens and striated muscle had already been broken and precipitated a break in tolerance to the acetylcholine receptor resulting in the development of MG. This case demonstrates the importance of prompt recognition of IL-2-induced MG and shows how this complication can be successfully managed with aggressive therapy.     

Efficient transfection of MG‐63 osteoblasts using magnetic nanoparticles and oscillating magnetic fields

Aims: To examine the potential of magnetic nanoparticles (MNPs) in transfecting human osteosarcoma fibroblasts (MG‐63) and investigate the effects of a novel non‐viral oscillating nanomagnetic gene transfection system (magnefect‐nano?) in enhancing transfection efficiency (TE). Methods: MG‐63 cells were transfected using MNPs coupled with a GFP‐carrying plasmid. The magnefect‐nano system was evaluated for transfection efficiency and potential associated effects on cell viability. Results: MG‐63 cells were efficiently transfected using MNPs and the magnefect‐nano system significantly enhanced overall transfection efficiency. MNPs were not found to affect cell viability and/or function of the cells. Conclusion: Non‐viral transfection using MNPs and the magnefect‐nano system can be used to transfect MG‐63 cells and assist reporter gene delivery on a single cell basis, highlighting the wide potential of nanomagnetic gene transfection in gene therapy. Copyright © 2012 John Wiley & Sons, Ltd.