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下呼吸道淀粉样变性。21例患者的临床及病理特征

Amyloidosis of the lower respiratory tract. Clinical and pathologic features in a series of 21 patients.

作者信息

Cordier J F, Loire R, Brune J

出版信息

Chest. 1986 Dec;90(6):827-31. doi: 10.1378/chest.90.6.827.

Abstract

Twenty one cases of amyloidosis of the lower respiratory tract were seen at a single center. In three patients, multifocal bronchial amyloid plaques led to stenosis and atelectasis, and in two, small pseudotumor masses were an incidental bronchoscopic finding. Two patients had nodular parenchymal amyloidosis, in one of whom the lesions were progressive and in the other static. Fifteen patients had diffuse parenchymal amyloidosis. Two of these had severe interstitial involvement and died in respiratory failure; eight had congestive cardiac failure, and parenchymal amyloidosis was a post-mortem finding; two had senile cardiorespiratory amyloidosis, also found at autopsy; and in three, the amyloidosis was associated with malignancy. The degree of respiratory embarrassment seemed to be related to the amount of amyloid in the gas diffusion zones, irrespective of the etiology of amyloidosis.

摘要

在一个中心共发现21例下呼吸道淀粉样变性病例。3例患者中,多灶性支气管淀粉样斑块导致狭窄和肺不张,2例患者经支气管镜检查偶然发现小的假瘤样肿块。2例患者有结节性实质淀粉样变性,其中1例病变呈进行性,另1例病变静止。15例患者有弥漫性实质淀粉样变性。其中2例有严重的间质受累,死于呼吸衰竭;8例有充血性心力衰竭,实质淀粉样变性为尸检结果;2例有老年性心肺淀粉样变性,也为尸检时发现;3例淀粉样变性与恶性肿瘤有关。呼吸窘迫的程度似乎与气体扩散区淀粉样物质的量有关,而与淀粉样变性的病因无关。

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