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儿童期肺出血肾炎综合征:血浆置换及免疫抑制治疗

Goodpasture's syndrome in childhood: treatment with plasmapheresis and immunosuppression.

作者信息

McCarthy L J, Cotton J, Danielson C, Graves V, Bergstein J

机构信息

Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis 46202-5283.

出版信息

J Clin Apher. 1994;9(2):116-9. doi: 10.1002/jca.2920090204.

Abstract

Goodpasture's syndrome rarely affects children. Therefore, we present our experience in a young boy whose pulmonary hemorrhage was dramatically resolved by three plasma exchanges. We believe the hemorrhage was caused primarily by acute capillaritis. He received cytoxan and steroids and a series of plasma exchanges which removed/suppressed his anti-glomerular basement membrane (anti-GBM) antibody production. However, after a year, his renal function did not return, and he required renal transplantation and continues to do well.

摘要

古德帕斯彻综合征很少影响儿童。因此,我们介绍一名小男孩的治疗经历,其肺部出血通过三次血浆置换得到显著缓解。我们认为出血主要由急性毛细血管炎引起。他接受了环磷酰胺和类固醇治疗以及一系列血浆置换,这些措施清除/抑制了他抗肾小球基底膜(抗GBM)抗体的产生。然而,一年后,他的肾功能并未恢复,需要进行肾移植,目前情况良好。

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