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β2-微球蛋白相关淀粉样变性

Beta-2-microglobulin-associated amyloidosis.

作者信息

Floege J, Ehlerding G

机构信息

Department of Medicine, Division of Nephrology, Hannover Medical School, Germany.

出版信息

Nephron. 1996;72(1):9-26. doi: 10.1159/000188801.

Abstract

beta2-Microglobulin-associated amyloidosis has emerged as a major complication of long-term renal replacement therapy. The syndrome is confined to those patients on nontransplant modes of therapy. It does not occur in patients with a functioning renal transplant or, if already present, it does not progress any further in such patients. In the population of ESRD patients on dialysis, beta2-microglobulin-associated amyloidosis affects most patients treated for more than 15 years and is a cause of significant morbidity and in rare cases even mortality. The present review, which is based on the presentation of a typical case, discusses the current knowledge on the pathogenesis, clinical manifestations, diagnosis, prevention and therapy of beta2-microglobulin-associated amyloidosis.

摘要

β2微球蛋白相关淀粉样变性已成为长期肾脏替代治疗的主要并发症。该综合征仅限于接受非移植治疗方式的患者。在有功能的肾移植患者中不会发生,或者如果已经存在,在这类患者中也不会进一步发展。在接受透析的终末期肾病患者群体中,β2微球蛋白相关淀粉样变性影响大多数接受治疗超过15年的患者,是导致显著发病甚至在罕见情况下导致死亡的原因。本综述基于一个典型病例的介绍,讨论了目前关于β2微球蛋白相关淀粉样变性的发病机制、临床表现、诊断、预防和治疗的知识。

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