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青少年型重症肌无力的免疫抑制治疗

Immunosuppressive treatment for juvenile myasthenia gravis.

作者信息

Badurska B, Ryniewicz B, Strugalska H

机构信息

Department of Neurology, Medical School Polish Academy of Sciences, Warsaw.

出版信息

Eur J Pediatr. 1992 Mar;151(3):215-7. doi: 10.1007/BF01954388.

Abstract

The result of immunosuppressive treatment in 20 cases of generalized myasthenia gravis with onset before age 16 were analysed. The patients age at the beginning of immunosuppressive treatment ranged from 10 to 22 years. Thirteen patients suffered from a severe form of myasthenia gravis with respiratory attacks, the remaining 7 demonstrated a moderately severe form with bulbar symptoms dominating the clinical picture. Indication for immunosuppressive treatment was lack of or only slight improvement after thymectomy. Prednisone was used in 17 cases, high dosage of methylprednisolone in 8 (in one case twice), azathioprine in 11 and cyclophosphamide in 10. Best results were obtained with cyclophosphamide since significant improvement was recorded in 14/20 of patients. Corticosteroids, i.e. prednisone and high-dose methylprednisolone proved to be of limited value.

摘要

对16岁前发病的20例全身性重症肌无力患者的免疫抑制治疗结果进行了分析。免疫抑制治疗开始时患者年龄在10至22岁之间。13例患者患有重症肌无力的严重形式,伴有呼吸发作,其余7例表现为中度严重形式,球部症状在临床表现中占主导地位。免疫抑制治疗的指征是胸腺切除术后缺乏改善或仅有轻微改善。17例使用泼尼松,8例使用高剂量甲泼尼龙(1例使用两次),11例使用硫唑嘌呤,10例使用环磷酰胺。环磷酰胺取得了最佳效果,因为20例患者中有14例有显著改善。皮质类固醇,即泼尼松和高剂量甲泼尼龙,证明价值有限。

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