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24 - Cystic Fibrosis Liver Disease

from SECTION IV - METABOLIC LIVER DISEASE

Published online by Cambridge University Press:  18 December 2009

By
Andrew P. Feranchak M.D.
Edited by
Frederick J. Suchy ,
Ronald J. Sokol  and
William F. Balistreri
Andrew P. Feranchak M.D.
Affiliation:
Assistant Professor, Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas; Department of Pediatric Gastroenterology and Hepatology, Children's Medical Center of Dallas, Dallas, Texas
Frederick J. Suchy
Affiliation:
Mount Sinai School of Medicine, New York
Ronald J. Sokol
Affiliation:
University of Colorado, Denver
William F. Balistreri
Affiliation:
University of Cincinnati
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Summary

Cystic fibrosis (CF) is a genetic disorder characterized by epithelial electrolyte transport abnormalities, elevated sweat Cl− concentrations, pancreatic insufficiency, and chronic lung disease in most patients. It is the most common potentially fatal genetic disorder in the Caucasian population, affecting 1 in 2400–3500 live births [1, 2]. It is an autosomal recessive disorder caused by a mutation in the gene for the cystic fibrosis transmembrane conductance regulator (CFTR), a membrane channel protein. The clinical significance of hepatobiliary disease in CF has not been well characterized primarily because of two factors: (1) pulmonary involvement leads to early mortality in a majority of patients, and (2) the clinical identification of CF-associated liver disease has been difficult because, although it is progressive, liver involvement is often asymptomatic until the appearance of end-stage complications. Recently, with improved pulmonary treatments, median life expectancy now exceeds 30 years [3] and CF-associated hepatobiliary disease is recognized and characterized more comprehensively. Liver disease is now the second major cause of death in CF [4]. In recent years, advances in our understanding of the function of CFTR in bile duct epithelia have provided a stronger scientific basis for the pathogenesis of the disease, leading to insights concerning potentially novel therapeutic approaches.

The earliest reports of CF, probably date to the Middle Ages with reports of malnourished and “sickly” children that tasted “salty” when kissed [5].

Type
Chapter
Information
Liver Disease in Children , pp. 572 - 594
Publisher: Cambridge University Press
Print publication year: 2007

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  • Cystic Fibrosis Liver Disease
    • By Andrew P. Feranchak, M.D., Assistant Professor, Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas; Department of Pediatric Gastroenterology and Hepatology, Children's Medical Center of Dallas, Dallas, Texas
  • Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
  • Book: Liver Disease in Children
  • Online publication: 18 December 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511547409.026
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  • Cystic Fibrosis Liver Disease
    • By Andrew P. Feranchak, M.D., Assistant Professor, Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas; Department of Pediatric Gastroenterology and Hepatology, Children's Medical Center of Dallas, Dallas, Texas
  • Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
  • Book: Liver Disease in Children
  • Online publication: 18 December 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511547409.026
Available formats
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To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

  • Cystic Fibrosis Liver Disease
    • By Andrew P. Feranchak, M.D., Assistant Professor, Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas; Department of Pediatric Gastroenterology and Hepatology, Children's Medical Center of Dallas, Dallas, Texas
  • Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
  • Book: Liver Disease in Children
  • Online publication: 18 December 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511547409.026
Available formats
×