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[骨佩吉特病——临床方面、诊断与治疗的当前综述]

[Paget's disease of bone-a current review of clinical aspects, diagnostics and treatment].

作者信息

Klemm P, Dischereit G, von Gerlach S, Lange U

机构信息

Abt. Rheumatologie, Klinische Immunologie, Osteologie und Physikalische Medizin, Campus Kerckhoff der Justus-Liebig Universität Gießen, Benekestr. 2-8, 61231, Bad Nauheim, Deutschland.

Rheumatologische Schwerpunktpraxis Marburg, Marburg, Deutschland.

出版信息

Z Rheumatol. 2021 Feb;80(1):48-53. doi: 10.1007/s00393-020-00897-x. Epub 2020 Oct 1.

Abstract

Paget's disease is a monostotic or polyostotic progressive skeletal disease with a genetic predisposition. The affected bone areas show osseous swelling and often grotesque deformation, chronic pain and fractures. Many cases are asymptomatic for a long time resulting in a late diagnosis. The pathogenesis is still unknown. In addition to a genetic predisposition, viral factors are also discussed. Laboratory tests and imaging are used for diagnosis. The effective principle of medicinal bisphosphonate treatment is to inhibit osteoclastic bone resorption and should be initiated early to prevent secondary complications. This article presents the current knowledge about this rare osteological disease.

摘要

佩吉特病是一种具有遗传易感性的单骨型或多骨型进行性骨骼疾病。受累骨区域出现骨质肿胀,常伴有奇形怪状的变形、慢性疼痛和骨折。许多病例长期无症状,导致诊断延迟。其发病机制尚不清楚。除遗传易感性外,病毒因素也在讨论之中。实验室检查和影像学检查用于诊断。药用双膦酸盐治疗的有效原理是抑制破骨细胞骨吸收,应尽早开始以预防继发性并发症。本文介绍了关于这种罕见骨病的当前知识。

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