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胆囊缺如

Agenesis of the gallbladder.

作者信息

Kabiri Hamed, Domingo Orville H, Tzarnas Chris D

机构信息

Department of Surgery, Mercy Catholic Medical Center, Philadelphia, Pennsylvania, USA.

出版信息

Curr Surg. 2006 Mar-Apr;63(2):104-6. doi: 10.1016/j.cursur.2005.04.018.

Abstract

Agenesis of the gallbladder, with normal bile ducts, is a rare congenital condition occurring in 13 to 65 people out of a population of 100,000 and probably results from a failure of the gallbladder bud to develop or vacuolize in utero. Reports of a familial tendency toward this condition may suggest screening of asymptomatic family members and speed treatment when symptoms manifest, thus avoiding surgery. Choledocholithiasis in patients with gallbladder agenesis is rare, the stones found are usually small, and patients had invariably first presented with jaundice. The authors describe a case of a 23-year-old patient with gallbladder agenesis and a large choledochal calculus and without jaundice at presentation. A review of the literature is presented.

摘要

胆囊缺如且胆管正常是一种罕见的先天性疾病,每10万人中有13至65人发病,可能是由于胆囊芽在子宫内发育失败或空泡化所致。有关于这种疾病家族倾向的报道可能提示对无症状家庭成员进行筛查,并在症状出现时加快治疗,从而避免手术。胆囊缺如患者发生胆总管结石很罕见,所发现的结石通常较小,且患者最初总是以黄疸为首发症状。作者描述了一例23岁胆囊缺如且有一枚大的胆总管结石的患者,该患者就诊时无黄疸。并对相关文献进行了综述。

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