Lung Transplantation and Survival in Children with Cystic Fibrosis

Theodore G. Liou, Frederick R. Adler, David R. Cox, and Barbara C. Cahill.

This study investigated the effects of lung transplantation on the survival and quality of life in children with cystic fibrosis. A total of 248 of the 514 children on the waiting list underwent lung transplantation in the United States during the period from 1992 through 2002. Proportional-hazards modeling identified four variables besides transplantation that were associated with changes in survival.The contributions of infection, diabetes, age on survival are discussed. The authors conclude that there is clearly improved survival for only 5 of 514 patients on the waiting list for lung transplantation. Prolongation of life by means of lung transplantation should not be expected in children with cystic fibrosis. A prospective, randomized trial is needed to clarify whether and when patients derive a survival and quality-of-life benefit from lung transplantation.

New England Journal of Medicine, 2007, 357, 2143-2152

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