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伴有正常骨代谢生物标志物的活动性 Pagets 骨病:病例报告及文献复习。

Active Paget's disease of bone with normal biomarkers of bone metabolism: a case report and review of the literature.

机构信息

Department of Endocrinology, University Hospital of Ioannina, Dim. Hatzi 68, Ioannina, 45445, Greece.

出版信息

Clin Rheumatol. 2011 Jan;30(1):139-44. doi: 10.1007/s10067-010-1579-z. Epub 2010 Oct 1.

Abstract

Paget's disease of bone is a focal skeletal disorder characterized by the formation of structurally abnormal bone, skeletal deformities, and other complications leading to bone pain and significant disability. It can involve one or more areas in a single bone (monostotic) or multiple bones (polyostotic). Most of the time the disease is asymptomatic and the diagnosis is made incidentally by increased levels of bone metabolism markers, especially alkaline phosphatase and is confirmed by specific findings in radiographs and radionuclide bone scan. In this report, we describe the case of a 65-year-old female with clinical and radiological findings of active Paget's disease of bone, but with absence of abnormal biochemical markers. The patient was given a dose of 5 mg zoledronic acid intravenously with significant clinical improvement within the next 6 months. The present case not only shows that Paget's disease of bone can occur in the setting of normal markers of bone metabolism but also that, in such cases, the response to treatment can be monitored by improvement in the clinical picture or by correct evaluation of the imaging findings.

摘要

佩吉特病是一种局部骨骼疾病,其特征为形成结构异常的骨骼、骨骼畸形和其他导致骨痛和严重残疾的并发症。它可以涉及单个骨骼的一个或多个区域(单发性)或多个骨骼(多发性)。大多数时候,该疾病无症状,通过骨代谢标志物水平升高(特别是碱性磷酸酶)偶然诊断,通过 X 线和放射性核素骨扫描的特定发现得到确认。在本报告中,我们描述了一例 65 岁女性的病例,其具有活跃的佩吉特病的临床和影像学表现,但无异常生化标志物。患者给予 5 毫克唑来膦酸静脉内给药,在接下来的 6 个月内临床状况显著改善。本病例不仅表明佩吉特病可以在骨代谢标志物正常的情况下发生,而且在这种情况下,通过临床症状的改善或通过对影像学发现的正确评估,可以监测治疗反应。

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