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伴有多种泌尿系统表现的家族性中空内脏肌病。

Familial hollow visceral myopathy with varying urological manifestations.

作者信息

Higman D, Peters P, Stewart M

机构信息

Department of Surgery, Joyce Green Hospital, Dartford.

出版信息

Br J Urol. 1992 Oct;70(4):435-8. doi: 10.1111/j.1464-410x.1992.tb15804.x.

Abstract

A family with hereditary hollow visceral myopathy is described, with characteristic variation in expression between affected members. Very severe and widespread involvement of the gastrointestinal and urinary tracts in 1 patient contrasted with isolated urinary tract involvement in 2 others, and it is suggested that hollow visceral myopathy should be considered in the differential diagnosis of primary detrusor failure. The management of urinary tract involvement is discussed and a conservative approach is recommended.

摘要

本文描述了一个患有遗传性中空内脏肌病的家族,患病成员之间存在特征性的表达差异。1例患者出现了非常严重且广泛的胃肠道和泌尿道受累,而另外2例仅表现为孤立的泌尿道受累,提示在原发性逼尿肌功能衰竭的鉴别诊断中应考虑中空内脏肌病。文中讨论了泌尿道受累的治疗方法,并推荐采用保守治疗。

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