J Korean Soc Transplant.
2002 Jun;16(1):126-132.
Pediatric Liver Transplantation for Metabolic Liver Disease
- Affiliations
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- 1Department of Surgery, Hallym University, Seoul, Korea. slee@hallym.or.kr
- 2Department of Surgery, Division of Transplantation, University of Michigan, USA.
Abstract
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PURPOSE: Metabolic liver disease may progress to liver cirrhosis or fulminant hepatic failure. Liver transplantation has been indicated in children with metabolic liver disease as an established curative treatment modality.
METHODS
Between October 1986 and December 1999, 139 pediatric liver transplantations were performed in 119 patients. Twenty nine liver transplantations were underwent in 24 patients (16 males/8 females, mean age +/-SD: 8.2+/-5.6 yr, ranging 3 months-17 yrs) for metabolic liver disease (20.2%). Of 24 patients, 3 cases (12.5%) with primary hyperoxaluria type 1 underwent combined liver/kidney transplantations. Multiple liver transplantations were performed in 3 patients (1 patient: 3 times, 2 patients: 2 times). Twenty five orthotopic liver transplants were performed in 22 patients (91.7%), while 3 cases of auxiliary heterotopic liver transplants were done in 2 patients (8.3%). The mean follow-up period was 48.2+/-37.2 months (ranging 6-133 months).
RESULTS
Patient and graft survival in metabolic disease at 1 year were 91.5%, 89.1%, in non-metabolic disease, 74.1%, 71.2%, respectively. Twelve patients (50%) suffered at least one episode of acute rejection after transplantation. The one-year-patient survival of rejection group was 91.7%, non-rejection group, 91.7%. Mortality developed in 6 patients (25%) after transplantation. The causes of death were sepsis (n=3), hepatic failure (n=2) and chronic rejection (n=1).
CONCLUSION
The result of liver transplantation in metabolic liver disease seems to be better than non-metabolic liver disease (p>0.05). No difference was found between rejection and non-rejection group in one-year-patient survival (91.7% vs. 91.7%).
