Liver transplantation in children with metabolic disorders in the United States

Liise K. Kayler, Cynthia S. Rasmussen, Dawn M. Dykstra, Jeffrey D. Punch, Steven M. Rudich, John C. Magee, Martin A. Maraschio, Juan D. Arenas, Darrell A. Campbell, Robert M. Merion

Research output: Contribution to journalArticle

37 Citations (Scopus)

Abstract

We studied pediatric liver transplantation for metabolic disease in a large national cohort to determine whether smaller studies suggesting a survival advantage for these recipients could be corroborated. We also hoped to determine whether higher survival rates in recipients with metabolic disease are associated with lack of structural liver disease, and to evaluate these recipients' risk factors for mortality. Data from the Scientific Registry of Transplant Recipients were used to analyze nationwide results (1990-99) of pediatric liver transplantation for patients with biliary atresia and metabolic disease. Adjusted patient survival rates for children with metabolic disease at 1 and 5years were 94% and 92%, respectively, - significantly higher than for recipients with biliary atresia (90% and 86%) (p=0.008). Cox regression models identified recipient black race [relative risk (RR)=5.1] and simultaneous transplantation of other organs (RR=3.2) as significant risk factors for mortality in the metabolic group. Adjusted survival rates for metabolic patients with structural and nonstructural liver diseases were similar to each other at both 1 and 5 years. Children with metabolic disease had significantly higher adjusted short- and long-term post-transplant survival rates than those with biliary atresia. Structural disease was not a risk factor for worse outcomes.

Original languageEnglish (US)
Pages (from-to)334-339
Number of pages6
JournalAmerican Journal of Transplantation
Volume3
Issue number3
DOIs
StatePublished - Mar 2003

Fingerprint

Metabolic Diseases
Liver Transplantation
Biliary Atresia
Survival Rate
Liver Diseases
Pediatrics
Mortality
Organ Transplantation
Proportional Hazards Models
Registries
Transplants
Survival

Keywords

  • Liver transplantation
  • Metabolic disorders
  • Outcomes
  • Pediatric

ASJC Scopus subject areas

  • Immunology

Cite this

Kayler, L. K., Rasmussen, C. S., Dykstra, D. M., Punch, J. D., Rudich, S. M., Magee, J. C., ... Merion, R. M. (2003). Liver transplantation in children with metabolic disorders in the United States. American Journal of Transplantation, 3(3), 334-339. https://doi.org/10.1034/j.1600-6143.2003.00058.x

Liver transplantation in children with metabolic disorders in the United States. / Kayler, Liise K.; Rasmussen, Cynthia S.; Dykstra, Dawn M.; Punch, Jeffrey D.; Rudich, Steven M.; Magee, John C.; Maraschio, Martin A.; Arenas, Juan D.; Campbell, Darrell A.; Merion, Robert M.

In: American Journal of Transplantation, Vol. 3, No. 3, 03.2003, p. 334-339.

Research output: Contribution to journalArticle

Kayler, LK, Rasmussen, CS, Dykstra, DM, Punch, JD, Rudich, SM, Magee, JC, Maraschio, MA, Arenas, JD, Campbell, DA & Merion, RM 2003, 'Liver transplantation in children with metabolic disorders in the United States', American Journal of Transplantation, vol. 3, no. 3, pp. 334-339. https://doi.org/10.1034/j.1600-6143.2003.00058.x
Kayler, Liise K. ; Rasmussen, Cynthia S. ; Dykstra, Dawn M. ; Punch, Jeffrey D. ; Rudich, Steven M. ; Magee, John C. ; Maraschio, Martin A. ; Arenas, Juan D. ; Campbell, Darrell A. ; Merion, Robert M. / Liver transplantation in children with metabolic disorders in the United States. In: American Journal of Transplantation. 2003 ; Vol. 3, No. 3. pp. 334-339.
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