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一位经产妇的Buschke-Ollendorff综合征:病例报告及文献综述

Buschke-Ollendorff syndrome in a grande multipara: a case report and short review of the literature.

作者信息

Al Attia H M, Sherif A M

机构信息

Department of Internal Medicine and Orthopaedic Surgery, Mafraq Hospital, Abu Dhabi, United Arab Emirates.

出版信息

Clin Rheumatol. 1998;17(2):172-5. doi: 10.1007/BF01452270.

Abstract

A 36-year-old short-statured grande multipara (gravida 10, para 8) with diabetes mellitus and hyperlipidaemia was incidentally found to have Buschke-Ollendorff syndrome (osteopoikilosis and dermatofibrosis lenticularis disseminata). The pelvis and hips, followed by the knees, were the sites mainly affected by the osteopoikilosis. The lumbosacral spine was also affected. She had a single connective tissue naevus on the right thigh. Apart from two abortions, the repeated pregnancies were uneventful and all her deliveries were normal at full-term or nearly full-term. With regard to the relationship between Buschke-Ollendorff syndrome and multiparity, it was apparent that multiparity did not affect the involved weight bearing bony structures, nor did the disseminated osteopoikilosis interfere with the endurance of multiple pregnancies. Buschke-Ollendorff syndrome is known generally to have a benign course, a feature that is illustrated in this case, even when associated with the stresses of multiparity. The report also provides a short and updated review of various clinical aspects of the syndrome and its associations, some of which are of a serious nature.

摘要

一名36岁身材矮小的经产妇(孕10产8),患有糖尿病和高脂血症,偶然发现患有Buschke - Ollendorff综合征(骨斑点症和播散性豆状皮肤纤维瘤病)。骨盆和髋部,其次是膝盖,是骨斑点症主要累及的部位。腰骶椎也受到影响。她右大腿有一个单一的结缔组织痣。除了两次流产外,多次怀孕均顺利,所有分娩均为足月或接近足月的正常分娩。关于Buschke - Ollendorff综合征与经产之间的关系,显然经产并不影响受累的负重骨骼结构,播散性骨斑点症也不干扰多次怀孕的耐受性。Buschke - Ollendorff综合征通常病程良性,本病例即说明了这一特征,即使与经产带来的压力相关时也是如此。该报告还对该综合征的各种临床方面及其关联进行了简短且最新的综述,其中一些关联性质严重。

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