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中国儿童重症肌无力

Myasthenia gravis in Chinese children.

作者信息

Chan-Lui W Y, Leung N K, Lau T T

出版信息

Dev Med Child Neurol. 1984 Dec;26(6):717-24. doi: 10.1111/j.1469-8749.1984.tb08163.x.

Abstract

A clinical study of 50 Southern Chinese myasthenic children observed for periods of two to 18 years (six years on average) revealed manifestations different from those of caucasian patients. Onset was early, at an average of 4.8 years. 82 per cent had ocular myasthenia. Ophthalmoplegia followed ptosis between three months and 10 years later. Additional facial and isolated limb-muscle fatigability developed in only 6 per cent within three months to 3 1/2 years. Only 12 per cent developed generalized myasthenia. Although extension from ocular to the generalized form did not occur later than 20 months after onset, a deterioration in ocular symptoms, without extension into generalized myasthenia, occurred in nine of 18 children during adolescence. Ptosis and generalized myasthenia responded better to anticholinesterase and/or prednisone. Ophthalmoplegia was difficult to treat. The natural clinical course was benign. Spontaneous remission occurred in 62 per cent of cases, but 54.8 per cent of these relapsed, all confined to ocular muscles. Although there was no familial occurrence of myasthenia gravis, an association was found between myasthenia and thyroid disorders in some patients and their relatives. The association with HLA BW46 antigen was striking. Acetylcholine receptor antibodies were absent in the majority, but mildly elevated titres were found in three of five patients whose ocular symptoms deteriorated during adolescence, without extension into generalized myasthenia.

摘要

一项针对50名中国南方患重症肌无力儿童的临床研究,观察期为2至18年(平均6年),结果显示其症状表现与白种人患者不同。发病较早,平均年龄为4.8岁。82%的患儿患有眼肌型重症肌无力。睑下垂后3个月至10年出现眼肌麻痹。仅6%的患儿在3个月至3年半内出现额外的面部及孤立肢体肌肉疲劳。仅12%的患儿发展为全身型重症肌无力。尽管从眼肌型发展为全身型不晚于发病后20个月,但18名儿童中有9名在青春期出现眼肌症状恶化,未发展为全身型重症肌无力。睑下垂和全身型重症肌无力对抗胆碱酯酶和/或泼尼松反应较好。眼肌麻痹难以治疗。其自然临床病程为良性。62%的病例出现自发缓解,但其中54.8%复发,均局限于眼肌。尽管重症肌无力无家族发病情况,但在一些患者及其亲属中发现重症肌无力与甲状腺疾病有关联。与HLA - BW46抗原的关联显著。大多数患者乙酰胆碱受体抗体阴性,但5名青春期眼肌症状恶化且未发展为全身型重症肌无力的患者中有3名抗体滴度轻度升高。

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