Hepatobiliary Disease in Cystic Fibrosis

Author:

Roy C. C.1,Weber A. M.1,Morin C. L.1,Lepage G.1,Brisson G.1,Yousef I.1,Lasalle R.1

Affiliation:

1. Pediatric Research Center Hôpital Ste‐Justine Departments of Pediatrics and Biochemistry I'Université de Montréal Montreal Quebec Canada

Abstract

SummaryThe 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.

Publisher

Wiley

Cited by 4 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Towards a Standardized Classification of the Hepatobiliary Manifestations in Cystic Fibrosis (CFHBI): A Joint ESPGHAN/NASPGHAN Position Paper;Journal of Pediatric Gastroenterology and Nutrition;2024-01

2. Assessment of Hepatic Function in Cystic Fibrosis by Lidocaine Metabolism;Journal of Pediatric Gastroenterology and Nutrition;2001-04

3. Vitamin A Concentration in the Liver Decreases with Age in Patients with Cystic Fibrosis;Journal of Pediatric Gastroenterology and Nutrition;1997-03

4. Hepatopulmonary Syndrome in a Child with Cystic Fibrosis;Journal of Pediatric Gastroenterology and Nutrition;1996-11

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