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Auxiliary Partial Orthotopic Liver Transplantation for Crigler-Najjar Syndrome Type I

Rela, Mohamed FRCS; Muiesan, Paolo MD; Vilca-Melendez, Hector MD; Dhawan, Anil MD; Baker, Alaster MRCP; Mieli-Vergani, Giorgina PhD; Heaton, Nigel David FRCS

Original Articles

Objective To determine if auxiliary partial orthotopic liver transplantation (APOLT) has the long-term potential to correct the underlying abnormality in Crigler-Najjar syndrome type 1 (CNS1) without the need for total liver replacement.

Background Orthotopic liver transplantation has been used successfully to replace the defective enzyme in CNS1. Experimental studies have shown that only 1% to 2% of the normal hepatocyte mass is needed for bilirubin conjugation. If APOLT corrects the underlying metabolic abnormality, it has the advantage of preserving the native liver, which would serve as a "safety net" should the graft fail, and there is the potential for gene therapy in the future with possible withdrawal of immunosuppression.

Methods Seven APOLT procedures were performed in six recipients with CNS1. Median age at transplantation was 10.5 years. Six transplants were performed as a left auxiliary liver transplant, and one was performed as a right auxiliary liver transplant. Median serum bilirubin level at transplantation was 320 μmol/L. All patients required 12 to 16 hours of phototherapy daily before the transplant to maintain serum bilirubin levels between 250 and 350 μmol/L.

Results Median serum bilirubin level was 50 μmol/L at day 5 after the transplant and 23 μmol/L at a median follow-up of 32 months. In four children, early severe acute rejection developed, requiring conversion to tacrolimus; one underwent a second transplant for chronic rejection and graft atrophy but died from lymphoproliferative disease 6 months after the second transplant.

Conclusions This report shows that APOLT is technically feasible and provides adequate hepatocyte mass to correct the underlying metabolic abnormality in CNS1.

From the Liver Transplant Surgical Service and Department of Child Health, King's College Hospital, London, United Kingdom

Correspondence: Nigel David Heaton, Liver Transplant Surgical Service, King's College Hospital, Denmark Hill, London SE5 9RS, United Kingdom.

Accepted for publication November 5, 1998.

© 1999 Lippincott Williams & Wilkins, Inc.