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The Murine Choline-Deficient, Ethionine-Supplemented (CDE) Diet Model of Chronic Liver Injury
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Published on: October 21, 2017

Hepatic complications of cystic fibrosis

H T Psacharopoulos, E R Howard, B Portmann

    Lancet (London, England)
    |July 11, 1981
    PubMed

    Insights

    Cystic fibrosis patients frequently develop liver disease, including cirrhosis and variceal bleeding. Aspirin should be avoided, and injection sclerotherapy may be preferred for managing bleeding in cystic fibrosis liver disease.

    Area of Science:

    • Pediatric Gastroenterology
    • Hepatology
    • Pulmonology

    Background:

    • Cystic fibrosis (CF) is a multisystem genetic disorder.
    • Liver involvement is a significant complication in CF patients.
    • Hepatic manifestations range from abnormal liver function tests to cirrhosis.

    Purpose of the Study:

    • To prospectively assess liver involvement in pediatric cystic fibrosis patients.
    • To evaluate the incidence and progression of liver disease in CF.
    • To identify optimal management strategies for variceal bleeding in CF.

    Main Methods:

    • Prospective observational study of 74 CF patients (aged 1-19 years) over 1-7 years.
    • Assessment included liver function tests, imaging for cirrhosis, and monitoring for complications like variceal bleeding and hypersplenism.
    • Treatment outcomes for variceal bleeding (shunt surgery vs. injection sclerotherapy) were compared.

    Main Results:

    • 20 patients presented with hepatic problems; 18 developed cirrhosis (age 4-13 years).
    • Variceal bleeding occurred in 6 patients, with 50% linked to aspirin use; aspirin avoidance is recommended.
    • Injection sclerotherapy demonstrated efficacy and better tolerance compared to shunt surgery for variceal bleeding control.

    Conclusions:

    • Liver disease, including cirrhosis and variceal bleeding, is a common and serious complication in cystic fibrosis.
    • Aspirin ingestion should be strictly avoided in CF patients due to the risk of precipitating variceal bleeding.
    • Injection sclerotherapy represents a promising and well-tolerated alternative for managing variceal bleeding in CF, potentially surpassing traditional shunt surgery.

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