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[在一名无肺部病变的年轻成人中,阑尾黏液嵌塞揭示黏多糖贮积症合并肝硬化]

[Appendicular mucoid impaction disclosing mucoviscidosis with cirrhosis in a young adult without pulmonary lesion].

作者信息

Glibert A, Adler M, Engelholm L, Vanderelst A, Cremer M

机构信息

Service de Gastroentérologie, Hôpital Erasme, Bruxelles.

出版信息

Gastroenterol Clin Biol. 1988 May;12(5):493-6.

PMID:3402694
Abstract

A 22-year-old man presented with pain in the right iliac fossa. Clinical examination suggested appendicitis and showed splenomegaly. Echography and abdominal CT-sca suggested the diagnosis of cystic fibrosis based on the association of signs of cirrhosis and pancreatic atrophy. The sweat test was positive. The hypothesis of a mucoid appendicular impaction with spontaneous regression was retained based on clinical and radiological signs. This atypical presentation of cystic fibrosis underscores the frequency of obstructive intestinal which occasionally reveals the disease in the adult, and on the absence in this case of otherwise frequently associated problems such as significant pulmonary disease and malnutrition.

摘要

一名22岁男性因右下腹疼痛就诊。临床检查提示阑尾炎,并发现脾肿大。超声检查和腹部CT扫描基于肝硬化和胰腺萎缩的体征关联提示囊性纤维化的诊断。汗液试验呈阳性。基于临床和影像学表现,考虑为黏液性阑尾阻塞伴自发消退。囊性纤维化的这种非典型表现凸显了成人肠梗阻的发生率,肠梗阻偶尔会揭示该疾病,且在本例中不存在其他常见相关问题,如严重肺部疾病和营养不良。

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