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表现为小肠梗阻的巨指纤维脂肪瘤病

Macrodactylia fibrolipomatosis presenting as a small bowel obstruction.

作者信息

Kenkare Sonya, Ainapurapu Bujji

机构信息

Department of Medicine, University of Illinois at Chicago College of Medicine, Chicago, IL, USA.

出版信息

South Med J. 2010 Mar;103(3):248-9. doi: 10.1097/SMJ.0b013e3181ce0e36.

Abstract

A patient presented with progressive abdominal distention, discomfort, nausea, vomiting, and constipation. The patient also had congenital macrodactyly of the third, fourth, and fifth digits of the left hand. He [corrected] was diagnosed with macrodactylia fibrolipomatosis with multiple small bowel lipomas and complete small bowel obstruction. Macrodactylia fibrolipomatosis is a rare condition characterized by benign fibrofatty infiltration involving the soft tissues of the distal arm or leg with associated dactylomegaly of the associated digits. This patient had a rare presentation that included extensive small bowel lipomatosis.

摘要

一名患者出现进行性腹胀、不适、恶心、呕吐和便秘。该患者左手第三、四、五指还患有先天性巨指症。他被诊断为伴有多发小肠脂肪瘤和完全性小肠梗阻的纤维脂肪性巨指症。纤维脂肪性巨指症是一种罕见疾病,其特征为良性纤维脂肪浸润累及上肢或下肢远端的软组织,并伴有相关手指的巨指症。该患者有罕见的表现,包括广泛的小肠脂肪瘤病。

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