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肺出血肾炎综合征和特发性肺含铁血黄素沉着症中的肺泡毛细血管基底膜病变。

Alveolar capillary basement membrane lesions in Goodpasture's syndrome and idiopathic pulmonary hemosiderosis.

作者信息

Donald K J, Edwards R L, McEvoy J D

出版信息

Am J Med. 1975 Nov;59(5):642-9. doi: 10.1016/0002-9343(75)90225-9.

Abstract

An ultrastructural study of lung distinguised between lesions of the alveolar capillary basement membrane in a case of Good pasture's syndrome and in three cases of idiopathic pulmonary hemosiderosis. In Goodpasture's syndrome, diffuse vascular injury with wide endothelial gaps, diffusely fragmented basement membranes and an electron dense layer on the basement membrane was found. In idiopathic pulmonary hemosiderosis, focal ruptures of the basement membrane were associated with hydropic changes in pneumocytes and, although fibroblasts were not seen, collagen deposition occurred within the basement membrane. immunofluorescent studies failed to show deposition of immunoglobulins G (IgG), A (IgA), M (IgM) or C3 in the lung in either disease. The ultrastructural lesions appear to separate these clinically similar entities.

摘要

一项超微结构研究对一例古德帕斯彻综合征患者及三例特发性肺含铁血黄素沉着症患者的肺泡毛细血管基底膜病变进行了区分。在古德帕斯彻综合征中,发现存在弥漫性血管损伤,内皮间隙增宽,基底膜弥漫性断裂以及基底膜上有电子致密层。在特发性肺含铁血黄素沉着症中,基底膜的局灶性破裂与肺细胞的水肿性改变相关,尽管未见到成纤维细胞,但基底膜内有胶原沉积。免疫荧光研究未能显示这两种疾病的肺部有免疫球蛋白G(IgG)、A(IgA)、M(IgM)或C3沉积。超微结构病变似乎可区分这些临床症状相似的疾病。

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