| Sumario: | Cystic fibrosis (CF) is an inherited autosomal recessive disease that affects exocrine systems including sinuses, lungs, pancreas, liver, skin, gastrointestinal tract, and reproductive tract. The effects on the liver ranges from elevated liver function tests to cirrhosis and liver failure. The laboratory and radiographic screening, treatment alternatives, and organ transplantation are important aspects in the care of CF patients. Liver transplant and lung-liver transplant can improve the quality of life and life expectancy. Optimal timing for referral for liver transplantation remains controversial. Future research of cystic fibrosis liver disease continues to focus on the CFTR defect, genetic modifiers, and the biliary bicarbonate exchange.
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