Liver transplantation in children with cystic fibrosis: A long-term longitudinal review of a single center's experience

Jonathan A. Fridell, Geoffrey J. Bond, George V. Mazariegos, David M. Orenstein, Ashokkumar Jain, Rakesh Sindhi, Jonathan Finder, Ernesto Molmenti, Jorge Reyes

Research output: Contribution to journalArticle

71 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1152-1156
Number of pages5
JournalJournal of pediatric surgery
Volume38
Issue number8
DOIs
StatePublished - Aug 1 2003

Fingerprint

Cystic Fibrosis
Liver Transplantation
Survival
End Stage Liver Disease
Lung Diseases
Allografts
Mortality
Morbidity
Lung
Liver
Incidence
Sputum
Sepsis
Therapeutics
Pediatrics
Population

All Science Journal Classification (ASJC) codes

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

Fridell, J. A., Bond, G. J., Mazariegos, G. V., Orenstein, D. M., Jain, A., Sindhi, R., ... Reyes, J. (2003). Liver transplantation in children with cystic fibrosis: A long-term longitudinal review of a single center's experience. Journal of pediatric surgery, 38(8), 1152-1156. https://doi.org/10.1016/S0022-3468(03)00260-4

Liver transplantation in children with cystic fibrosis : A long-term longitudinal review of a single center's experience. / Fridell, Jonathan A.; Bond, Geoffrey J.; Mazariegos, George V.; Orenstein, David M.; Jain, Ashokkumar; Sindhi, Rakesh; Finder, Jonathan; Molmenti, Ernesto; Reyes, Jorge.

In: Journal of pediatric surgery, Vol. 38, No. 8, 01.08.2003, p. 1152-1156.

Research output: Contribution to journalArticle

Fridell, JA, Bond, GJ, Mazariegos, GV, Orenstein, DM, Jain, A, Sindhi, R, Finder, J, Molmenti, E & Reyes, J 2003, 'Liver transplantation in children with cystic fibrosis: A long-term longitudinal review of a single center's experience', Journal of pediatric surgery, vol. 38, no. 8, pp. 1152-1156. https://doi.org/10.1016/S0022-3468(03)00260-4
Fridell, Jonathan A. ; Bond, Geoffrey J. ; Mazariegos, George V. ; Orenstein, David M. ; Jain, Ashokkumar ; Sindhi, Rakesh ; Finder, Jonathan ; Molmenti, Ernesto ; Reyes, Jorge. / Liver transplantation in children with cystic fibrosis : A long-term longitudinal review of a single center's experience. In: Journal of pediatric surgery. 2003 ; Vol. 38, No. 8. pp. 1152-1156.
@article{ad57c9ab37574a5bb0951538c107bf97,
title = "Liver transplantation in children with cystic fibrosis: A long-term longitudinal review of a single center's experience",
abstract = "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.",
author = "Fridell, {Jonathan A.} and Bond, {Geoffrey J.} and Mazariegos, {George V.} and Orenstein, {David M.} and Ashokkumar Jain and Rakesh Sindhi and Jonathan Finder and Ernesto Molmenti and Jorge Reyes",
year = "2003",
month = "8",
day = "1",
doi = "10.1016/S0022-3468(03)00260-4",
language = "English (US)",
volume = "38",
pages = "1152--1156",
journal = "Journal of Pediatric Surgery",
issn = "0022-3468",
publisher = "W.B. Saunders Ltd",
number = "8",

}

TY - JOUR

T1 - Liver transplantation in children with cystic fibrosis

T2 - A long-term longitudinal review of a single center's experience

AU - Fridell, Jonathan A.

AU - Bond, Geoffrey J.

AU - Mazariegos, George V.

AU - Orenstein, David M.

AU - Jain, Ashokkumar

AU - Sindhi, Rakesh

AU - Finder, Jonathan

AU - Molmenti, Ernesto

AU - Reyes, Jorge

PY - 2003/8/1

Y1 - 2003/8/1

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=0041859729&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0041859729&partnerID=8YFLogxK

U2 - 10.1016/S0022-3468(03)00260-4

DO - 10.1016/S0022-3468(03)00260-4

M3 - Article

C2 - 12891484

AN - SCOPUS:0041859729

VL - 38

SP - 1152

EP - 1156

JO - Journal of Pediatric Surgery

JF - Journal of Pediatric Surgery

SN - 0022-3468

IS - 8

ER -