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肺出血肾炎综合征的眼部临床发现及基底膜变化

Ocular clinical findings and basement membrane changes in Goodpasture's syndrome.

作者信息

Jampol L M, Lahov M, Albert D M, Craft J

出版信息

Am J Ophthalmol. 1975 Mar;79(3):452-63. doi: 10.1016/0002-9394(75)90622-4.

Abstract

Goodpasture's syndrome is a rapidly progressive disease characterized by the onset of pulmonary hemorrhage with glomerulonephritis, leading to pulmonary and renal failure. The hallmark of this disease is linear deposition of antibasement membrane IgG in the basement membranes of the kidney and lung. In two cases of nonrhegmatogenous retinal detachment in patients with Goodpasture's syndrome, the first patient demonstrated multiple ischemic areas in the choroid on fluorescein angiography. Histopathologic and immunologic studies of the eyes in the second patient demonstrated macular edema and elevation of the retina in association with choroidal infarction and linear deposition of IgG in Burch's membrane and the basement membranes of the choroidal vessels.

摘要

古德帕斯彻综合征是一种快速进展性疾病,其特征为伴有肾小球肾炎的肺出血发作,可导致肺和肾衰竭。该疾病的标志是抗基底膜IgG在肾脏和肺的基底膜中呈线性沉积。在两例古德帕斯彻综合征患者发生的非孔源性视网膜脱离病例中,首例患者在荧光素血管造影检查中显示脉络膜多处缺血区。对第二例患者眼睛进行的组织病理学和免疫学研究显示,黄斑水肿、视网膜隆起,伴有脉络膜梗死以及IgG在伯奇膜和脉络膜血管基底膜中的线性沉积。

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