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棕色肠综合征(肠道脂褐质沉着症)——病例报告及文献综述

Brown bowel syndrome (intestinal lipofuscinosis) - a case report and review of the literature.

作者信息

Białas Magdalena, Demczuk Sergiusz, Dyduch Grzegorz, Drabik Grażyna, Chrupek Małgorzata, Okoń Krzysztof

机构信息

Magdalena Białas, MD, Department of Pathomorphology, ul. Grzegórzecka 16, 31-351 Kraków, tel. +48 12 421 15 64, e-mail:

出版信息

Pol J Pathol. 2013 Oct;64(3):228-31. doi: 10.5114/pjp.2013.38144.

Abstract

Brown bowel syndrome (BBS) is a very rare condition occurring in association with malabsorption syndromes. It is characterised by deposition of granular, brown pigment (lipofuscin or ceroid) in intestinal smooth muscles. Rarely BBS can be complicated by distention of any segment of the bowel. We present a case of BBS associated with massive dilation of first loop of the small intestine and moderate dilation of the sigmoid colon with functional intestinal disturbances requiring surgical intervention in an 11-year-old boy.

摘要

棕色肠综合征(BBS)是一种与吸收不良综合征相关的非常罕见的病症。其特征是颗粒状棕色色素(脂褐素或类蜡质)沉积于肠道平滑肌。BBS很少会并发肠道任何节段的扩张。我们报告一例11岁男孩的BBS病例,该病例伴有小肠第一袢的大量扩张和乙状结肠的中度扩张,并伴有功能性肠道紊乱,需要进行手术干预。

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