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%).
Cause of Death ; Child ; Female ; Follow-Up Studies ; Graft Survival ; Humans ; Hyperoxaluria, Primary ; Liver Cirrhosis ; Liver Diseases* ; Liver Failure ; Liver Failure, Acute ; Liver Transplantation* ; Liver* ; Metabolic Diseases ; Mortality ; Sepsis