Hepatobiliary Disease in Cystic Fibrosis

作者：Claudie Roy, Alexander Mark Weber, C L Morin, Guy Lepage, G. R. Brisson, Ibrahim M. Yousef, R. Lasalle · 发表于：Journal of Pediatric Gastroenterology and Nutrition · 年份：1982 · DOI：10.1097/00005176-198212000-00005 · 被引用次数：99 · 研究领域：Cystic Fibrosis Research Advances、Pediatric Hepatobiliary Diseases and Treatments、Iron Metabolism and Disorders

The incidence of hepatobiliary complications of cystic fibrosis (CF) has been increasing in parallel with the rate of survival. Detection of hepatic involvement remains a problem, as liver function tests, serum bile acid determinations, and ultrasonography do not permit an early diagnosis. The pathogenesis of cholelithiasis has been elucidated in the past few years. However, the mechanism leading to the pathognomonic CF lesion, focal biliary cirrhosis, is still unknown. There are indications that mucus plugging may be the ancestral lesion and the triggering factor. The possibility that correction of the abnormalities of bile acid metabolism could slow the progress or prevent CF cirrhosis is discussed in light of recent experimental data.