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Liver Disorders

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Cystic Fibrosis

An inherited genetic condition of the exocrine glands, cystic fibrous (CF) is associated with many systemic diseases, including the liver. Often referred to as hepatobiliary disease, CF-related liver disease appears to be increasing, affecting adults who live with CF in their 30s, 40s and 50s. Conditions can include hepatotoxicity, biliary obstructions, biliary cirrhosis and nutritional deficiencies.

Five to 20 percent of newborns with CF develop neonatal cholestasis (impaired bile flow), which can mimic extrahepatic biliary atresia or coincide with a fat-soluble deficiency. Some child and teens have elevated liver enzymes; others remain symptom free. In adults over 24-years-old, focal biliary cirrhosis is more common, presenting as jaundice, hypersplenism or an abnormal intraperitoneal build up of fluid.

Diagnosis includes physical exam of the spleen and liver, liver ultrasound, endoscopic retrograde cholengiopancreatography to detect biliary obstructions, and bile flow evaluation. Treatment includes medical therapy and liver transplant in select patients.