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 Vol. 19 No. 4, April 2008
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AAP Grand Rounds 19:41-42 (2008)
© 2008 American Academy of Pediatrics

PULMONOLOGY

Lung Transplants in Children with Cystic Fibrosis

Source: Liou TG, Adler FR, Cox DR, et al. Lung transplantation and survival in children with cystic fibrosis. N Engl J Med. 2007;357(21):2143–215; doi:10.1056/NEJMoa066359[Abstract/Free Full Text]

The first 20% of the full text of this article appears below.


PICO

Question: In children age 18 years or younger with cystic fibrosis and end-stage lung disease, does lung transplantation improve survival?

Question type: Therapy

Study design: Retrospective review

 

To determine whether lung transplantation prolongs life for children with cystic fibrosis (CF), investigators from the University of Utah and Nuffield College, UK used data from the US Cystic Fibrosis Foundation Patient Registry (CFFPR) and from the Organ Procurement and Transplantation Network (OPTN) to identify children with CF placed on the candidate waiting list for lung transplantation from 1992 through 2002.

The CFFPR includes longitudinal data on children from 117 certified CF Centers, while the OPTN includes information for all patients listed as candidates for lung transplantation in the US. Patient-specific identifiers were used to match patients in the two data sets.

The study population consisted of 514 children with CF listed for lung transplantation in the US during the study period for whom complete data were available. Two hundred forty-eight children (48%) received a lung transplant. The median survival time for children who died before transplantation was 223 days and the median time to transplantation was 427 days. One hundred twenty children died after transplantation; 60% of the deaths were due to complications of transplantation (primarily allograft rejection). The median length of survival after transplantation was 1,037 days (2.8 . . . [Full Text of this Article]

George Mallory, MD, FAAP
Baylor School of Medicine, Texas Children’s Hospital, Houston, TX

 





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