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淀粉样变性

Amyloidosis.

作者信息

Husby G

机构信息

Department of Rheumatology, University and Regional Hospital of Tromsø, Norway.

出版信息

Semin Arthritis Rheum. 1992 Oct;22(2):67-82. doi: 10.1016/0049-0172(92)90001-t.

Abstract

Amyloidosis is a heterogenous group of diseases characterized by deposition of a fibrillar, proteinaceous material, amyloid, in various tissues and organs. Increasing knowledge about the different proteins that constitute the amyloid fibrils has made it possible to classify amyloidosis by the fibril protein, which appears more rational than the traditional classification by its clinical expression. A serum protein is the precursor of the amyloid fibril protein in the various systemic forms of amyloidosis. Although the chemical composition of amyloid is presently well known, the pathogenetic processes that convert such proteins into a fibrillar form and lay them down in the tissues are far from clarified. This review describes the amyloid deposits, some putative pathogenetic mechanisms, and the clinical, therapeutic, and prognostic aspects of the most important forms of amyloid disease.

摘要

淀粉样变性是一组异质性疾病,其特征是一种纤维状蛋白质物质——淀粉样蛋白沉积于各种组织和器官中。对构成淀粉样纤维的不同蛋白质的认识不断增加,使得根据纤维蛋白对淀粉样变性进行分类成为可能,这似乎比传统的按临床表现分类更为合理。在各种系统性淀粉样变性中,一种血清蛋白是淀粉样纤维蛋白的前体。尽管目前淀粉样蛋白的化学成分已为人熟知,但将此类蛋白质转化为纤维状并沉积于组织中的致病过程仍远未阐明。本综述描述了淀粉样蛋白沉积、一些可能的致病机制以及最重要形式的淀粉样疾病的临床、治疗和预后方面。

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