The Child with Cystic Fibrosis Liver Disease

  • Deirdre Kelly


Cystic fibrosis (CF) is the most common life limiting autosomal recessive disorder of Caucasians. The defect in the cystic fibrosis transmembrane regulator (CFTR) protein causes an inability to maintain normal hydration of luminal tracts which leads to thickened secretions and obstruction. As survival with lung disease improves, the recognition of liver pancreas and bowel disease is increasing. Liver disease develops in a third of CF patients and causes 2.5% of CF deaths (Table 8.1). It is more common in male CF patients and those with meconium ileus as a neonate.

Further Reading

  1. Debray D, Kelly D, Houwen R, Strandvik B, Colombo C. Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. J Cyst Fibros. 2011;10(Suppl 2):S29–36.CrossRefPubMedGoogle Scholar
  2. Smyth AR, Bell SC, Bojcin S, Bryon M, et al. European cystic fibrosis society standards of care: best practice guidelines. J Cyst Fibros. 2014;13(Suppl 1):S23–42.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.The Liver UnitBirmingham Women’s and Children’s HospitalBirminghamUK

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