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[古德帕斯彻综合征]

[Goodpasture's syndrome].

作者信息

Herout V, Nozicka Z, Erben J

出版信息

Cesk Patol. 1978 Nov;14(4):186-94.

PMID:367611
Abstract
  1. The paper summarizes the clinical course, histopathological examinations and immunohistological examinations of 4 personal cases [2 males aged 20 and 26 years, 2 females aged 65 and 18 years] of Goodpasture's pulmorenal syndrome. 2. The characteristic histopathological findings included pulmonary haemorrhage with a focal acute alveolitis and renal lesions that were classed as acute or subacute glomerulonephritis with the finding of scarce multinucleated cells. Such cells are regarded as being of diagnostic value and they are thought to indicate interaction of antibodies with the basement membranes. 3. Immunohistologically, all the cases showed a linear fluorescence following the basement membrane of the interalveolar septa. In 3 of the cases there was linear fluorescence in the glomerular capillary walls. In one case [observation No. 2] who had been hypertensive before death and had had necrotizing arteriitis at postmortem, there was fluorescence of granular type in the kidney believed to indicate the participation of immune complexes in the development of the pathological condition.
摘要
  1. 本文总结了4例Goodpasture肺肾综合征患者[2例男性,年龄分别为20岁和26岁,2例女性,年龄分别为65岁和18岁]的临床病程、组织病理学检查及免疫组织学检查结果。2. 特征性组织病理学表现包括肺出血伴局灶性急性肺泡炎以及肾脏病变,后者被归类为急性或亚急性肾小球肾炎,可见少量多核细胞。此类细胞被认为具有诊断价值,提示抗体与基底膜的相互作用。3. 免疫组织学检查显示,所有病例在肺泡间隔基底膜处均呈现线性荧光。3例病例在肾小球毛细血管壁出现线性荧光。1例病例[观察编号2]生前有高血压,尸检时有坏死性动脉炎,其肾脏出现颗粒型荧光,提示免疫复合物参与了病理状况的发生发展。

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[Goodpasture's syndrome].[古德帕斯彻综合征]
Cesk Patol. 1978 Nov;14(4):186-94.

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