Abstract
Between March 1984 and August 1994, 13 orthotopic liver transplantations were performed in 13 patients ≤ 25 years of age. The indications included Wilson's disease (n = 7), biliary atresia (n = 4), choledochal cyst (n = 1) and hepatitis C cirrhosis (n = 1). Technical variants included full-size (n = 11), reduced-size (n = 1) and living-related (n = 1) liver transplantation. These recent technical innovations have offered an expanded donor pool for earlier transplantation, shorter waiting times and excellent quality grafts. Surgical complications occurred in six patients; all required additional surgery. Biliary complications were encountered more commonly in our earlier patients. Our actuarial patient and graft survival rate is 92% at 2 years. The long-term follow-up of our liver-transplanted Wilson's disease patients provides confirmatory evidence that orthotopic liver transplantation cures the underlying metabolic defect with complete normalization of biochemical abnormalities of copper metabolism, reversal of neurological impairments and the disappearance of Kayser-Fleischer corneal rings. The high rate of patient survival and excellent rehabilitation indicate that with prudent clinical judgement, liver transplantation can be achieved with an acceptable rate of morbidity, mortality and cost in a setting where manpower and donor organs are very limited.
Original language | English |
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Pages (from-to) | S1-S3 |
Journal | Journal of Gastroenterology and Hepatology (Australia) |
Volume | 11 |
Issue number | 5 |
DOIs | |
State | Published - 1996 |
Externally published | Yes |
Keywords
- Biliary atresia
- Liver transplantation
- Paediatric
- Wilson's disease