LUNG TRANSPLANTATION FOR CYSTIC-FIBROSIS - EXPERIENCE OF UNIVERSITA-DI-ROMA-LA-SAPIENZA

Cystic fibrosis (CF) is the most common genetic disorder in the white population and although the disease involves several organs, about 90% of the patients die of pulmonary insufficiency. Lung transplantation may offer gook results in term of better survival and quality of life. At University of Roma <<La Sapiena>>, between November 1996 and Febru ary 1998, 13 patients with cystic fibrosis and pulmonary insufficiency were transplanted. Nine patients were female and 4 male with a mean a ge of 27 +/- 5,6 years. The mean forced expiratory volume in 1 second (FEV1) was 20,4 +/- 5% predicted. Bilateral sequential transplantation was performed. No intraoperative complications occurred. Two patients dies respectively after 15 and 45 days after transplantation. Eleven patients are alive and well after a mean follow-up of 10,5 months (ran ge from 5 to 16 months). Two patients developed complications of the b ronchial anastomosis successfully treated by laser and endobronchial s tents positioning. In 5 cases we observed early or delayed dehiscence of the surgical wound. Minor complications also occurred. All complica tions resolved with the appropriate therapy. The mean length of hospit al stay was 28,56 days, ranging between 19 and 36 days. Pulmonary func tion test improved dramatically as well as the quality of life. Until now, no patients present evidence of obliterative bronchiolitis. Our e xperience contributes to emphasize the importance of lung transplantat ion in this subset of patients.