PRIMARY BILIARY-CIRRHOSIS

被引：14

作者：

LAURIN, JM

LINDOR, KD

机构：

[1] Division of Gastroenterology, Mayo Clinic and Foundation, Rochester, MN

来源：

DIGESTIVE DISEASES | 1994年 / 12卷 / 06期

关键词：

PRIMARY BILIARY CIRRHOSIS; PYRUVATE DEHYDROGENASE COMPLEX; AUTOIMMUNE DISORDERS; URSODEOXYCHOLIC;

D O I：

10.1159/000171469

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

Primary biliary cirrhosis is a chronic, usually progressive, cholestatic liver disease of presumed autoimmune etiology that affects predominantly young and middle-aged women. It is nearly always associated with an antibody directed against a component of the pyruvate dehydrogenase complex located on the inner wall of the mitochondria. The disease is associated with a number of other associated autoimmune disorders. No totally effective medic;ll treatment has been established for the disease, although ursodeoxycholic acid appears promising. Complications of cholestasis such as fat malabsorption and fat-soluble vitamin deficiency should be excluded or corrected when found. Individual patient prognosis varies. Several models for estimating individual patient survival are available. Liver transplantation is recognized as a procedure to extend and improve the quality of life for patients with advanced disease.

引用

页码：331 / 350

页数：20

共 132 条

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