Liver transplantation in cystic fibrosis: Monitoring and followup - Personal experience

About 5-15% of children with cystic fibrosis develop hepatic cirrhosis and progressive portal hypertension. When the portal hypertension worsens these children are likely to develop serious variceal bleeding and other complications including malnutrition and a decline in respiratory function. Isolated liver transplantation is the therapy of choice in patients with cystic fibrosis affected by liver cirrhosis and initial clinical manifestations of portal hypertension in presence of a well preserved pulmonary function (REV1 > 40%). Although the optimal time for liver transplantation is difficult to judge this procedure provides a progressive improvement of patient respiratory function, nutritional status and quality of life, meanwhile not increasing the risk of lung infection due to immunosuppression.