重症肌无力的免疫发病机制及相关治疗进展

重症肌无力（MG）是一种自身免疫性疾患。主要发病机制是通过自身抗体的产生,导致肌肉运动终板乙酰胆碱受体（AchR）密度下降。研究证实,抗AchR抗体、肌肉特异性激酶（MuSK）抗体及多种针对其他肌肉胞浆蛋白的抗体与重症肌无力的发病相关。该病治疗主要采用胆碱酯酶抑制剂、肾上腺皮质类固醇或其他免疫抑制剂、胸腺切除术、血浆置换和免疫球蛋白等。应根据病人及疾病特点采取个体化的治疗方案,干细胞疗法为难治性MG提供了一条新的治疗。     

小儿哮喘免疫学发病机制进展

哮喘的主要病理过程为气道黏膜水肿,嗜酸性粒细胞、淋巴细胞和中性粒细胞浸润;气道内分泌物增多。在气道炎症的基础上,出现气道高反心性(BHR),发生广泛性细小支气管管腔狭窄或闭塞。血清和气道分泌物中总免疫球蛋白(IgE)和过敏原特异性IgE明显增高。这些病理和病理生理改变的本质是异常的免疫反应。     

重症肌无力免疫机制的研究进展

重症肌无力(MG)是一种依赖T细胞的乙酰胆碱受体抗体介导的自身免疫性疾病,其中自身抗体和细胞因子是MG免疫机制的研究重点。综述近年来对于这些方面的研究内容,发现有多种自身抗体的存在且作用不同,辅助T细胞1细胞因子如IL 2、IL 12、γ干扰素和肿瘤坏死因子 α可能在MG和实验性自动免疫性MG中起到了一定的作用,而细胞因子如转化生长因子 β、α干扰素可能有助于MG和实验性自动免疫性MG的形成;相反,辅助T细胞2细胞因子在MG中的作用仍不明了。     

重症肌无力免疫机制的研究进展

重症肌无力(MG)是一种依赖T细胞的乙酰胆碱受体抗体介导的自身免疫性疾病，其中自身抗体和细胞因子是MG免疫机制的研究重点。综述近年来对于这些方面的研究内容，发现有多种自身抗体的存在且作用不同，辅助T细胞1细胞因子如IL-2、IL-12、γ干扰素和肿瘤坏死因子-α可能在MG和实验性自动免疫性MG中起到了一定的作用，而细胞因子如转化生长因子-β、α干扰素可能有助于MG和实验性自动免疫性MG的形成；相反，辅助T细胞2细胞因子在MG中的作用仍不明了。     

支气管哮喘的免疫学发病机制

支气管哮喘(简称哮喘)是儿童期最常见的呼吸道慢性疾病之一,涉及各个年龄组近20年来,全球范围的的哮喘发病率和病死率均呈上升趋势,严重影响儿童身心健康和生活质量。探索哮喘的发病机制已成为世界研究的热点。     

过敏性紫癜免疫学发病机制研究进展

过敏性紫癜是儿童常见的自身免疫性小血管炎,其发病机制尚未完全明了.近年IgA介导的免疫学异常研究颇多,IgA沉积于受累肾小球系膜区及皮肤、肾小球、胃肠道血管壁,组织沉积原因可能涉及血清IgA产生增多、IgA特异性自身抗体异常、IgA1结构异常、IgA1受体表达异常、补体参与等,IgA的沉积介导黏附分子、细胞因子等表达异常.     

IgA血管炎免疫学发病机制的研究进展

IgA血管炎(IgAV)是儿童最常见的白细胞碎裂性小血管炎,主要累及皮肤小血管、关节、消化道及肾脏,其发病机制并不完全明确。目前认为是在遗传的基础上,受环境影响,引起自身免疫系统功能紊乱,IgA为主的免疫复合物在小动脉壁沉积而发病。该文主要探讨免疫因素在IgAV发病机制中的作用。     

系统性红斑狼疮的免疫学发病机制研究进展

系统性红斑狼疮是一种儿童常见的自身免疫性疾病,其免疫学的发病机制复杂.随着免疫学和基因组学的发展,目前的研究已发现其发病与T细胞及B细胞的免疫学异常,尤其是T、B细胞导致的自身免疫耐受被打破,从而产生致病性自身抗体有关,并且系统性红斑狼疮的发病与儿童原发性免疫缺陷密切相关.此外,固有免疫细胞的调节功能异常及其导致的外周免疫耐受的破坏与系统性红斑狼疮的发病及疾病进展有重要联系.     

系统性红斑狼疮的免疫学发病机制研究进展

系统性红斑狼疮是一种儿童常见的自身免疫性疾病,其免疫学的发病机制复杂.随着免疫学和基因组学的发展,目前的研究已发现其发病与T细胞及B细胞的免疫学异常,尤其是T、B细胞导致的自身免疫耐受被打破,从而产生致病性自身抗体有关,并且系统性红斑狼疮的发病与儿童原发性免疫缺陷密切相关.此外,固有免疫细胞的调节功能异常及其导致的外周免疫耐受的破坏与系统性红斑狼疮的发病及疾病进展有重要联系.     

Early-onset myasthenia gravis

Signs of myasthenia gravis developed by age 3 years in 11 children. Six of these patients had persistent neonatal myasthenia gravis, a familial abnormality of neuromuscular transmission that is not immunologically mediated. Five patients had juvenile onset myasthenia gravis, an autoimmune disorder similar to myasthenia gravis in adults. Autoimmune myasthenia has rarely been recognized by age 3 years, but the presence of five cases in our series suggests that the disorder may be more common in young children than once believed. The development of anti-acetylcholine receptor antibody assays makes it easier to distinguish autoimmune myasthenia gravis from the congenital forms. This distinction is important, because the prognosis, treatment, and risk of recurrence in family members is different for each type of myasthenia.     

Neonatal myasthenia gravis]

BACKGROUND: Myasthenia gravis, an autoimmune disease of young women, is due to the dysfunction of neuromuscular transmission. The newborn of a myasthenic mother inconstantly presents a transitory neonatal myasthenic syndrome. Maternal aggravation, or even myasthenic crisis with respiratory failure, can occur in the first three months post-partum. CASE REPORT: Mrs. S., para two without appreciable medical history, delivered normally a boy weighing 4 kg with an Apgar score of 10/10. At 3 h of life the newborn was admitted to the neonatal care unit for grunting and axial hypotonia. Diagnoses of maternal-fetal infection and fetal distress were excluded. The dissociated pattern of neurological disorders (refusal to drink, axial hypotonia, hypomimia, but good contact and normal alertness) led to search for neuromuscular causes or poison. Myasthenia gravis was then considered and confirmed by maternal electromyography, allowing the diagnosis of transient neonatal myasthenia gravis and early diagnosis and treatment of the maternal myasthenic crisis in a specialized care unit. The outcome of both mother and child was favorable under treatment. CONCLUSION: Lack of maternal myasthenia gravis history should not result in excluding the diagnosis of transitory neonatal myasthenia gravis when evocative neonatal neurological signs are present. The symptomatology in the newborn may indeed reveal maternal myasthenia gravis, allowing an early diagnosis in both the mother and the newborn.     

The prognosis of juvenile myasthenia gravis

In 18 patients with juvenile myasthenia gravis (MG) the mean age of disease-onset was 12.1 years. The mean observation time was 6.8 years. After classification with regard to the clinical severity of the disease (Ossermann, 1958) 4 patients (22%) could be assigned to type I, 10 patients (56%) to type II A, 2 patients (11%) to type II B, and 2 patients (11%) to type III. 14 patients (77%) could be assigned to type I and type II A, both of them rather benign types. All 4 male patients belonged to this group. The tensilon test, investigated in 15 patients, showed a positive result in 13 cases (87%). The repetitive stimulation was done in 13 patients and was positive only in 6 cases (46%). Investigations in MG-patients without limitation of age showed positive results in about 70%. Increased levels of acetylcholin-receptor-antibody were found in 10 of 11 patients (91%). In 8 patients with the diagnosis MG, type II A and III, a thymectomy was done, 16 patients received cholinesterase-blockers, 1 patient with type II A, and both patients in group III received additionally azathioprin. In 1 patient with type III plasmapheresis was done. 75% of all thymectic patients showed a remission or improvement. Related to all 18 patients we found in 16 cases (89%) a remission or correction of the symptoms. In summary the prognosis in the group of our patients with juvenile myasthenia gravis was good.     

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??Abstract??Juvenile myasthenia gravis is not rare in china. Diagnosis in children can be complicated by the compliance of diagnostic tests and limited positive findings of auxiliary examination. We need to pay more attention to the differential diagnosis.The treatment of juvenile myasthenia gravis is not standardized?? including the option of treatment and the therapy of recurrence of myasthenia gravis. There is a small subset of patients?? however?? with treatment-refractory myasthenia gravis. The management of myasthenia should be undertaken.     

儿童重症肌无力的治疗

1　抗胆碱酯酶 (ＣｈＥ)药物 (ＣｈＥ抑制剂 )1933年Ｗａｌｋｅｒ应用抗ＣｈＥ药物治疗重症肌无力(ＭＧ) ,作用机制是化学结构类似乙酰胆碱 (Ａｃｈ) ,竞争性与ＣｈＥ的活性中心结合 ,使Ａｃｈ降解速度减慢 ,使神经肌肉接头处 (ＮＭＪ)Ａｃｈ量得以增多 ,从而使Ａｃｈ击中Ａｃｈ受体的机会增加[1] 。疗效肯定 ,但系对症治疗。1 1　新斯的明1 1 1　溴化新斯的明片剂 (每片 15ｍｇ)　 5岁以内0 5ｍｇ/ (ｋｇ·ｄ) ,5岁以上 0 2 5ｍｇ/ (ｋｇ·ｄ) ,每 4ｈ 1次 ,逐渐加量 ,一旦出现副作用则停止加量。 10～ 2 0ｍｉｎ生效 ,持续3～ 4ｈ ,极量…     

重症肌无力的治疗进展

重症肌无力(myasthenia gravis,MG)是乙酰胆碱受体抗体介导的自身免疫性疾病,随着对MG发病机制研究的深入,MG的治疗取得了巨大进展.该文从树突状细胞诱导的免疫耐受、单克隆抗体阻断、造血干细胞移植疗法等方面进行综述,以便为MG治疗提供依据.     

Respiratory form mof myasthenia gravis]

BACKGROUND. Extraocular, facial, bulbar and intercostal muscles are frequently affected in juvenile myasthenia gravis, especially during exacerbations. Acetylcholine receptor antibodies are often present in the blood in this type of myasthenic syndrome. CASE REPORT. A girl presented with an exertional dyspnea at the age of 13 years, that improved after rest. All investigations were negative, except for lung function tests that showed a restrictive pattern. The diagnosis of juvenile myasthenia gravis was finally made at the age of 15 years because of the recurrence of sudden exertional dyspnea and a history of subtle weakness on repetitive movement leading to poor suckling, together with vocal and occasional swallowing difficulties. Dyspnea immediately improved after intravenous injection of 1 mg neostigmine, but the response was transient. No acetylcholine receptor antibody was found and a search for thymoma proved negative. Ambenonium chloride (Mytelase) was effective on clinical exacerbations, but the only improved test after 9 months of treatment was the functional residual capacity. CONCLUSION. Anticholinesterase drugs must be tried in patients who present exertional dyspnea without bronchopulmonary or cardiac disease on the presumption of myasthenia gravis even when ocular or bulbar manifestations are absent.