Pediatric liver transplantation for metabolic liver disease: experience at King's College Hospital.
OBJECTIVE
The aims of this article were to report a single-center experience of pediatric liver transplantation for liver-based metabolic disorders and to compare the outcome of cirrhotic versus noncirrhotic metabolic liver disease.


METHODS
The medical records of 96 patients younger than 18 years undergoing transplantation for liver-based metabolic disorders from 1989 to 2005 were reviewed.


RESULTS
Hundred twelve transplants were performed in 96 patients at a median age of 59.7 months (range, 0-208 months). The cumulative 1-, 5-, and 10-year graft and patient survival rates were 83%, 77%, and 62% and 91%, 86%, and 82%, respectively. Acute liver failure at first presentation (hazard ratio [HR] 3.0; 95% confidence interval [CI] 1.1-8.1), age less than 1 year at time of transplantation (HR 4.6; 95% CI 1.7-12.4) and hospitalization (HR 3.2; 95% CI 1.1-9.3) were significant predictors of worse patient survival. For noncirrhotic disorders, the long-term patient (100% vs. 100%, 90% vs. 100%, and 90% vs. 75%, P=0.87) and graft survivals (93% vs. 100%, 70% vs. 100%, and 70 vs. 75%, P=0.12) at 1, 5, and 7 years for auxiliary versus orthotopic transplantation were not significantly different.


CONCLUSIONS
Long-term patient survival after transplantation for metabolic disorders is excellent for both cirrhotic and noncirrhotic metabolic disorders. For noncirrhotic metabolic disorders, auxiliary transplantation has similar patient and graft survival compared with orthotopic transplantation, but further research is recommended.
Sze
Yuk K
YK
Paediatric Liver Centre, King's College Hospital NHS Foundation Trust, London, United Kingdom.
Dhawan
Anil
A
Taylor
Rachel M
RM
Bansal
Sanjay
S
Mieli-Vergani
Giorgina
G
Rela
Mohammed
M
Heaton
Nigel
N
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005355:Fibrosis / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D005500:Follow-Up Studies; D006085:Graft Survival; D006761:Hospitals; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008107:Liver Diseases / Q000453:epidemiology* / Q000378:metabolism* / Q000473:pathology / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D024821:Metabolic Syndrome / Q000453:epidemiology* / Q000378:metabolism* / Q000473:pathology / Q000601:surgery; D015996:Survival Rate; D006113:United Kingdom