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Medical Therapy
CF patients with abnormal liver function typically take ursodeoxycholic acid (ursodiol) which decreases bile duct clogging and helps bile flow from the liver. Ursodiol often lowers abnormally elevated liver function tests. In patients with liver dysfunction, fat-soluble vitamin levels should be carefully monitored to ensure that supplements are adequate. Hepatitis A and B vaccinations should be given, too. Patients with liver disease should avoid alcohol and acetaminophen, which can worsen liver damage. Any herbal supplements or over-the-counter remedies should be discussed with a physician. Liver Transplantation
Patients with severe liver disease may qualify for liver transplantation. Patients with mild lung disease and severe liver disease are the best candidates. Only 10 liver transplants are performed for CF patients in the United States each year. In children and young adults, the estimated five-year survival rate after transplant is approximately 75 percent. Patients who have undergone successful liver transplants usually have improved nutritional status and quality of life.
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