1: Lancet 1993 Sep 25;342(8874):779-80

Orthotopic auxiliary liver transplantation for Crigler-Najjar syndrome type 1

Whitington PF, Emond JC, Heffron T, Thistlethwaite JR
Department of Pediatrics, University of Chicago Pritzker School of Medicine,
Wyler Children's Hospital, IL

Abstract
Some diseases that result from inborn errors of critical metabolic or synthetic
processes mainly involving the liver do not cause structural liver damage. These
disorders can be treated by the addition of liver tissue (auxiliary liver
transplantation) rather than liver replacement. We report correction of the
metabolic error in a 13-year-old girl with Crigler-Najjar syndrome type 1 by
auxiliary (left lateral segment) transplantation. The first graft failed and was
replaced successfully. The second graft shows features of chronic rejection, but
at 2 years postoperatively bilirubin conjugating ability has not been impaired.
Another graft may become necessary in due course