Objectives: The aim of this study was to evaluate pulmonary function in four patients with cystic fibrosis (CF) after liver transplantation.
Patients and methods: From 1993 to 1997 three males and one female, aged 12 to 15 years, required liver transplantation for CF with cirrhosis and portal hypertension. Three had a history of esophageal variceal bleeding. In three patients, forced vital capacity (FVC) and forced expiratory volume in one second (FEV1) before liver transplantation were over 80 and 75% of predicted values, respectively; in the fourth patient FVC was 37% and FEV1was 26%. Two patients presented allergic bronchopulmonary aspergillosis before transplantation. Only one patient was chronically infected in sputum with multiresistant Pseudomonas aeruginosa and none had Burkholderia cepacea.
Results: After liver transplantation, only the patient with P. aeruginosa in sputum culture and the worst pulmonary function presented a complicated course requiring mechanical ventilation for 43 days followed by non-invasive nasal ventilation for 8 months. This patient died 19 months after transplantation. The remaining three patients, with better pulmonary function before transplantation, presented an uncomplicated course and currently lead normal lives.
Conclusions: We conclude that liver transplantation can improve pulmonary function and is well tolerated in children with CF and mild or moderate pulmonary involvement. When pulmonary involvement is severe, combined lung and liver transplantation should be considered.