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Additional data on hepatic function tests in cystic fibrosis.

J Feigelson, Y Pecau, L Cathelineau

    Acta Paediatrica Scandinavica
    |March 1, 1975
    PubMed
    Summary
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    This study observed 50 cystic fibrosis (CF) patients for 3 years, finding distinct biochemical patterns in those with cirrhosis. Liver function tests revealed abnormalities, distinguishing cholestatic and cellular cirrhosis types.

    Area of Science:

    • Hepatology
    • Gastroenterology
    • Pediatric Medicine

    Background:

    • Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, including the liver.
    • Liver disease, specifically cirrhosis, can be a complication in CF patients.
    • Understanding the biochemical profiles of liver complications in CF is crucial for patient management.

    Purpose of the Study:

    • To investigate liver function and biochemical patterns in cystic fibrosis patients.
    • To differentiate between various types of liver cirrhosis in CF patients.
    • To assess the prevalence and characteristics of liver abnormalities in CF.

    Main Methods:

    • Longitudinal observation of 50 cystic fibrosis patients over 3 years.
    • Performance of liver function tests assessing cytolysis, cholestasis, and cellular insufficiency.

    Related Experiment Videos

  • Assay of immunoglobulin and prothrombin levels.
  • Analysis of IgA:Transferrin ratio and gamma-glutamyl-transpeptidase levels for cirrhosis subtyping.

    • Main Results:

    • Nine out of 50 CF patients developed multilobular cirrhosis, showing generally abnormal liver function tests.
    • Two distinct biochemical patterns of cirrhosis were identified: cholestatic and cellular.
    • The IgA:Transferrin ratio and gamma-glutamyl-transpeptidase levels were key in distinguishing these patterns.
    • In non-cirrhotic CF patients, 50% exhibited temporary increases in cytolysis and cholestasis.

    Conclusions:

    • Cystic fibrosis patients with cirrhosis exhibit specific biochemical abnormalities.
    • Liver cirrhosis in CF can be biochemically classified into cholestatic and cellular types.
    • These biochemical distinctions aid in understanding and potentially managing liver complications in CF.