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β2微球蛋白淀粉样变性与肾性骨病

Beta-2-microglobulin amyloidosis and renal bone disease.

作者信息

Drüeke T B

机构信息

Unité 90 de l'INSERM, Hôpital Necker, Paris, France.

出版信息

Miner Electrolyte Metab. 1991;17(4):261-72.

PMID:1813789
Abstract

Dialysis-related amyloidosis is a highly invalidating complication of chronic dialysis patients. Clinically and radiologically, the complication generally becomes manifest after more than 5-10 years of dialysis treatment. It is characterized by chronic arthralgias, periarticular soft-tissue swelling, carpal tunnel syndrome, diffuse destructive arthropathy and spondylarthropathy, lytic bone lesions, and sometimes pathologic fractures. Biochemically, amyloid deposits are mainly composed of beta-2-microglobulin fibrils together with the P component and glucose aminoglycans, the latter two being associated with all types of amyloidosis. The pathogenesis of the syndrome is still poorly understood. Recently, evidence has been provided for the possibility that beta-2-microglobulin is not a passive bystander, but an active player in the initiation and progression of the disease. The potential role of the dialysis technique, including the mode of dialysis and the biocompatibility of dialysis membrane used, is also under discussion.

摘要

透析相关淀粉样变性是慢性透析患者一种极具致残性的并发症。在临床和放射学上,该并发症通常在透析治疗5至10年以上后才会显现。其特征为慢性关节痛、关节周围软组织肿胀、腕管综合征、弥漫性破坏性关节病和脊椎关节病、溶骨性骨病变,有时还会出现病理性骨折。在生物化学方面,淀粉样沉积物主要由β2微球蛋白原纤维以及P成分和葡萄糖氨基聚糖组成,后两者与所有类型的淀粉样变性有关。该综合征的发病机制仍知之甚少。最近,有证据表明β2微球蛋白并非被动旁观者,而是疾病发生和发展过程中的积极参与者。透析技术的潜在作用,包括透析方式和所用透析膜的生物相容性,也在讨论之中。

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