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Liver and biliary problems in cystic fibrosis
S G Williams1, D Westaby, M S Tanner
1Department of Gastroenterology, Charing Cross Hospital, London, UK.
British Medical Bulletin
|October 1, 1992
Summary
Biliary cirrhosis in cystic fibrosis patients is slowly progressive and often asymptomatic, though its long-term effects and optimal management require further research. Advances in treatment control complications, but new techniques and trials are needed to fully assess efficacy.
Area of Science:
- Hepatology
- Gastroenterology
- Pediatric Gastroenterology
Background:
- Biliary cirrhosis is a recognized complication in a minority of cystic fibrosis patients.
- Significant advancements in managing cirrhosis complications have occurred over 50 years.
- Many aspects of biliary cirrhosis in cystic fibrosis, including pathogenesis and optimal management, remain poorly understood.
Purpose of the Study:
- To summarize current knowledge on biliary cirrhosis in cystic fibrosis.
- To assess the efficacy of existing management strategies.
- To identify areas requiring further research and clinical trials.
Main Methods:
- Review of clinical and pathological studies.
- Analysis of epidemiological data.
- Evaluation of current treatment modalities for variceal bleeding and biliary complications.
Main Results:
- Cirrhosis in cystic fibrosis is often asymptomatic and slowly progressive, with prevalence increasing with age.
- Age-related prevalence may decrease in patients over 20, with rare deaths from liver disease.
- Current treatments effectively manage variceal bleeding, reducing morbidity and mortality.
Conclusions:
- Further research is needed to understand factors initiating liver disease and progressive fibrosis in cystic fibrosis.
- Newer endoscopic and radiological techniques offer adjuncts to surgical management of biliary complications.
- Ursodeoxycholic acid's effect on fibrosis and portal hypertension requires large-scale trials; the role of liver transplantation needs evaluation.