Lung transplantation is a common procedure with significant risks undertaken in children with cystic fibrosis. This retrospective study investigates the effect of lung transplantation on survival.

Five hundred and fourteen of 602 children (85%) aged <18 years with cystic fibrosis placed on the lung transplantation waiting list in the USA between 1992 and 2002 were included in the study; 248 of these children subsequently underwent lung transplantation. Proportional hazards modelling identified Burkholderia cepacia infection, diabetes, increasing age and Staphylococcus aureus infection as factors other than transplantation that had an impact upon survival. By also modelling transplantation as a time-dependent covariate, the authors calculated that 5 patients had a significant estimated benefit, 315 had a significant risk of harm, 76 had an insignificant benefit and 118 had an insignificant risk of harm associated with lung transplantation.

Although other factors that could not be measured in the study may have affected survival, the data and methods used seem to justify the conclusion of the authors that lung transplantation should not be undertaken in children with cystic fibrosis with the aim of prolongation of life expectancy. They acknowledge, however, that it remains to be determined whether, for some children, lung transplantation can be justified on quality of life grounds against an acceptable complication and mortality risk. A prospective randomised trial will be required to determine whether this is indeed the case. Until such information is available, it is questionable under what circumstances lung transplantation should be performed in children with cystic fibrosis.

Liou TG, Adler FR, Cox DR, et al. Lung transplantation and survival in children with cystic fibrosis. N Engl J Med 2007;357:2143–52