Severe late acute allograft rejection in a child after living-related auxiliary partial orthotopic liver transplantation for ornithine transcarbamylase deficiency

Auxiliary liver transplantation (ALT) is known to correct liver-based metab olic disorders. However, it remains unclear whether the presence of a nativ e liver influences the long-term prognosis of ALT for metabolic diseases. W e reported on a 4-yr-old girl who had undergone living-related auxiliary pa rtial orthotopic liver transplantation (APOLT) for ornithine transcarbamyla se deficiency and experienced severe late acute rejection 18 months after l iver transplantation, during weaning of immunosuppressive agents. Results o f histological analysis of the graft indicated very severe acute rejection (rejection activity index, 9/9), and computed tomography revealed graft liv er atrophy. These observations suggest the possibility that severe rejectio n might occur in APOLT, especially during weaning of immunosuppression.