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原发性气管支气管淀粉样变性。一例病例报告及已报道病例综述。

Primary tracheobronchial amyloidosis. A case report and review of reported cases.

作者信息

Wakasa K, Sakurai M, Koezuka I, Yoshida J, Oumi S, Monden Y, Tani Y

出版信息

Acta Pathol Jpn. 1984 Jan;34(1):145-52.

PMID:6375249
Abstract

A case of diffuse tracheobronchial amyloidosis in a 70-year-old woman thought clinically at first to have lung carcinoma is reported. Bronchoscopic biopsy was necessary to make a diagnosis. Amyloid was deposited markedly in the subepithelial layer. After Congo-red staining amyloid showed the usual apple-green birefringence under polarizing microscope. Electron microscopic study disclosed typical amyloid fibrillar structure. Amyloid protein of this patient was confirmed to be AL protein by immunoperoxidase study which showed specific staining by the anti-A lambda antiserum. When potassium permanganate reaction was used amyloid protein of this patient resisted to the reaction. We reviewed 19 cases from the previously reported cases of primary amyloidosis of the lower respiratory tract, and gave some description of pathologic and clinical aspects of this disease.

摘要

报道了一例70岁女性的弥漫性气管支气管淀粉样变病例,该患者最初临床诊断考虑为肺癌。需要进行支气管镜活检来确诊。淀粉样物质显著沉积于上皮下层。刚果红染色后,淀粉样物质在偏振显微镜下呈现出典型的苹果绿双折射。电子显微镜研究揭示了典型的淀粉样纤维结构。通过免疫过氧化物酶研究证实该患者的淀粉样蛋白为AL蛋白,抗Aλ抗血清显示出特异性染色。当使用高锰酸钾反应时,该患者的淀粉样蛋白对该反应有抗性。我们回顾了先前报道的19例下呼吸道原发性淀粉样变病例,并对该疾病的病理和临床方面进行了一些描述。

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