Liver transplantation in children with cystic fibrosis:

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August 2003 • Volume 38 • Number 8

Liver transplantation in children with cystic fibrosis: A long-term

longitudinal review of a single center’s experience

A. Fridella,c [MEDLINE LOOKUP]

   Abstract  TOP

Background: Improved long-term survival in cystic fibrosis (CF) has led

to an increased incidence of extrapulmonary complications of this

disease. Of these, end-stage liver disease is a significant cause of

morbidity and mortality with liver transplantation being the only

effective therapy. Methods: Records of all CF pediatric liver transplant

recipients were reviewed. Results: Twelve children with CF were the

recipients of 16 allografts. The 1- and 5-year survival was 91.6% and

75%, respectively. There were 5 deaths at a mean interval of 6.8 ± 6.3

years. All of these deaths were related to pulmonary disease. Pulmonary

function improved or remained stable in 8 of 9 patients tested. Despite

an 83% incidence of positive sputum cultures, there was only one early

mortality related to pulmonary sepsis in the setting of primary liver

allograft nonfunction. Conclusions: Liver transplantation is acceptable

treatment for children with CF and end-stage liver disease. Long-term

survival is comparable to liver transplantation performed for other

indications. Although posttransplant morbidity and mortality is related

to lung disease, the authors speculate that as therapeutic improvements

prolong the survival in CF, it is expected that longer survival after

liver transplantation in this patient population may also be anticipated.

   Publishing and Reprint Information  TOP

aDepartment of Pediatric Transplantation, E. Starzl

Transplantation Institute, Children’s Hospital of Pittsburgh, Pittsburgh,

PA USA

b J. and Janet Palumbo Cystic Fibrosis Center, Department of

Pediatrics, University of Pittsburgh Medical Center, Pittsburgh, PA; USA

cDepartment of Surgery, Indiana University School of Medicine,

Indianapolis, IN, USA

dDepartment of Surgery, The s Hopkins Hospital, Baltimore, MD, USA

*Address reprint requests to Reyes, MD, E. Starzl

Transplantation Institute, Children’s Hospital of Pittsburgh, Room 7

North, 7950, 3705 Fifth Ave, Pittsburgh, PA 15213, USA

Copyright © 2003 by Elsevier Inc.

Becki

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