原发性硬化性胆管炎可表现为急性肝衰竭：两例报告。

Primary sclerosing cholangitis can present with acute liver failure: Report of two cases.

作者信息

Bergquist Annika, Glaumann Hans, Lindberg Bo, Broomé Ulrika

机构信息

Department of Gastroenterology, Karolinska University Hospital, Huddinge, Karolinska Institute, Stockholm,

出版信息

J Hepatol. 2006 May;44(5):1005-8. doi: 10.1016/j.jhep.2006.01.033. Epub 2006 Feb 28.

DOI:10.1016/j.jhep.2006.01.033

PMID:16551483

Abstract

BACKGROUND/AIMS: The aim of the present study was to determine whether PSC can present with acute liver failure (ALF) and to determine its frequency.

METHODS

Medical records from all patients with a well-defined PSC (n=246), treated at Karolinska University Hospital, Huddinge between 1984 and 2004 were scrutinized. Information on PSC and inflammatory bowel disease (IBD) characteristics including mode of presentation of PSC was evaluated. A group of patients with ALF of indeterminate cause at our hospital diagnosed (1993-2003) was identified as a reference group (n=46).

RESULTS

Two patients with PSC presented with ALF (1%) and are described in detail. Both of them had an underlying IBD. Nobody in the reference group had IBD.

CONCLUSIONS

In conclusion, PSC patients can present with ALF in approximately 1% of all patients. PSC should be considered as a differential diagnosis in patients with ALF of indeterminate cause, especially in patients with IBD.

摘要

背景/目的：本研究的目的是确定原发性硬化性胆管炎（PSC）是否会表现为急性肝衰竭（ALF）并确定其发生率。