The Child with Cystic Fibrosis Liver Disease
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.