Liver transplantation in cystic fibrosis.
(2001) Current Opinion in Pulmonary Medicine — Vol. 7, n° 6, p. 441-447 (2001)
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- Authors
- Abstract
- Liver disease is the second most common cause of death in patients with cystic fibrosis (CF). Improvement in surgical techniques, medical management, and imaging modalities has broadened the range of options for treatment of these patients. Medical management with ursodeoxycholic acid and nutritional support may help decelerate the progression of liver disease. A timely evaluation of CF patients with liver involvement for transplantation is important. Such evaluation should not be delayed until signs of hepatic decompensation occur. Combined lung-liver transplant can be considered for patients with advanced pulmonary disease. Pretransplant management of portal hypertension with a portosystemic shunt procedure is an option for patients with well-preserved synthetic liver function. Improvement in lung function after liver transplantation and no significant risk of pulmonary infection with immunosuppressive therapy have been reported. Review of individual center experiences have shown satisfactory survival and improved quality of life for CF patients undergoing liver transplant.
- Affiliations
Keck School of Medicine, University of Southern California, University Hospital, Los Angeles, USA Division of Hepatobiliary/Pancreatic Surgery and Abdominal Organ Transplantation
Citations
Genyk, Y. S., Quiros, J. A., Jabbour, N., Selby, R. R., & Thomas, D. W. (2001). Liver transplantation in cystic fibrosis. Current Opinion in Pulmonary Medicine, 7(6), 441-447. https://hdl.handle.net/2078.5/189209 (Original work published 2001)