Auxiliary liver transplantation: a form of gene therapy in selective metabolic disorders

Abstract

Auxiliary liver transplantation is an accepted form of therapy in acute liver failure and in certain metabolic disorders. We report India's first successful auxiliary liver transplantation for Crigler-Najjar syndrome type 1, showing that it is technically feasible and safe procedure. It is utmost important to select appropriate cases for auxiliary transplant for successful long-term outcome. The surgeon should also have an understanding of the portal flow dynamics, as steal phenomenon can occur, depriving blood blow to either graft or native liver. Though successful in animal models, gene therapy is still in experimental stage in humans and pace of progress has been disappointing. Auxiliary liver transplantation retains the native liver for future gene therapy. These children are young and are likely to have a long life expectancy, and withdrawal of immunosuppression would be a huge advantage.

Keywords: ALF, acute liver failure; APOLT, auxiliary partial orthotopic liver transplants; Acute liver failure; CNS1, Crigler-Najjar syndrome type 1; Crigler-Najjar syndrome; auxiliary liver transplant; gene therapy; pediatric transplantation.