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囊性纤维化中的全身性淀粉样变性。

Generalized amyloidosis in cystic fibrosis.

作者信息

Michalsen H, Storrøsten O T, Lindboe C F

出版信息

Eur J Respir Dis. 1985 Apr;66(4):306-9.

PMID:4018182
Abstract

A boy suffering from cystic fibrosis (CF) complicated by generalized amyloidosis is reported. His condition was fairly good during the first 10 years of life, but after this time he had repeated pulmonary infections caused by Staphylococcus aureus. From the age of 14, he developed increasing hepato- and splenomegaly and a liver biopsy revealed massive amyloidosis. At the age of 16 he died of respiratory and cardiac failure. Post-mortem examination revealed wide-spread amyloidosis in addition to pulmonary and pancreatic findings characteristic of CF.

摘要

报告了一名患有囊性纤维化(CF)并伴有全身性淀粉样变性的男孩。他在生命的头10年情况相当良好,但此后他反复发生由金黄色葡萄球菌引起的肺部感染。从14岁起,他出现肝脾肿大且逐渐加重,肝脏活检显示有大量淀粉样变性。16岁时,他死于呼吸和心力衰竭。尸检发现除了有CF特有的肺部和胰腺表现外,还有广泛的淀粉样变性。

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