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肺出血肾炎综合征:一例尸检病例报告及日本病例综述。

Goodpasture's syndrome: a report of an autopsy case and a review of Japanese cases.

作者信息

Wakui H, Chubachi A, Asakura K, Nishimura S, Nakamoto Y, Miura A B

机构信息

Department of Internal Medicine, Yuri-Kumiai General Hospital, Honjo, Japan.

出版信息

Intern Med. 1992 Jan;31(1):102-7. doi: 10.2169/internalmedicine.31.102.

Abstract

A 75-yr-old man was admitted because of acute renal failure. On the 9th hospital day, pulmonary hemorrhage occurred. He was treated with a bolus dose of methylprednisolone and plasma exchange, but died from respiratory failure on the 16th hospital day. Autopsy findings demonstrated marked intraalveolar hemorrhage and crescentic glomerulonephritis. Linear depositions of IgG along both alveolar and glomerular basement membranes (GBM) were shown by direct immunofluorescent studies. Circulating anti-GBM antibodies were demonstrated by indirect immunofluorescent techniques. This is a confirmatory case of Goodpasture's syndrome, which is rare in Japan. A review of the Japanese literature revealed some characteristics of Japanese cases.

摘要

一名75岁男性因急性肾衰竭入院。住院第9天发生肺出血。给予大剂量甲泼尼龙和血浆置换治疗,但于住院第16天死于呼吸衰竭。尸检结果显示肺泡内有明显出血和新月体性肾小球肾炎。直接免疫荧光研究显示IgG沿肺泡和肾小球基底膜(GBM)呈线性沉积。间接免疫荧光技术检测到循环抗GBM抗体。这是一例确诊的古德帕斯彻综合征病例,该综合征在日本较为罕见。对日本文献的回顾揭示了日本病例的一些特征。

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