Lung Transplantation for Cystic Fibrosis in Italy

• Published in: Transplantation proceedings 2008-07, Vol.40 (6), p.2003-2005
• Main Authors: Quattrucci, S, Cimino, G, Bertasi, S, Benedetti Valentini, S, Bossi, A, D'Alu, V, Locorriere, L, Scacchi, C, Rolla, M
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; Bronchiolitis Obliterans - epidemiology; Cystic Fibrosis - complications; Cystic Fibrosis - surgery; Female; Fundamental and applied biological sciences. Psychology; Fundamental immunology; Humans; Italy; Lung Transplantation - statistics & numerical data; Male; Medical sciences; Pneumology; Postoperative Complications - classification; Postoperative Complications - epidemiology; Reoperation - statistics & numerical data; Respiratory Insufficiency - etiology; Respiratory Insufficiency - surgery; Respiratory system : syndromes and miscellaneous diseases; Retrospective Studies; Surgery; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Tissue, organ and graft immunology; Treatment Outcome
• ISSN: 0041-1345; 1873-2623
• DOI: 10.1016/j.transproceed.2008.05.036

Abstract Lung transplantation (LT) is the only effective form of therapy for cystic fibrosis (CF) associated with end-stage pulmonary failure. In Italy, the management of CF is regulated by national law, which has instituted regional centers for care and follow-up of all CF patients. LT has been performed since 1992 in only nine LT certified centers. The structured national organization has led to a unified database for LT for CF. As of December 2006, 197 bilateral LT (96 male and 94 female patients; 7 retransplants) have been performed. Of these, four had also liver or heart and liver transplantation, and three are long-term survivors. Overall median survival is 7 years. Mean age at transplantation is 26.5 years, and the mortality on the waiting list is 33.6%. Patients listed for transplant either received a suitable donor within a mean of 10 months or died within a mean of 5.5 months. The most frequent cause of death is bronchiolitis obliterans syndrome (BOS). Our nationwide database indicates the excellent results obtained by LT in FC. Still, mortality on the waiting list remains a challenge and long-term outcome is limited by BOS.