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P6.09 Long term outcomes of liver transplantation for metabolic defects. A single center report

Srinath Chinnakotla, United States

Professor of Surgery
University of MInnesota


Long term outcomes of liver transplantation for metabolic defects. A single center report

Heli Bhatt1, Varvara Kirchner1, David Vock1, SaraJane Schwarzenberg1, Srinath Chinnakotla1.

1Surgery/Pediatrics, University of Minnesota, Minneapolis, MN, United States

Liver transplantation (LT) was initially developed as a therapy for liver diseases known to be associated with a high risk of near-term mortality. As overall outcomes for the procedure have improved, LT has evolved into an attractive approach for a growing number of metabolic defects in a variety of   clinical situations.  Few studies have evaluated the long term outcomes of liver transplantation in this subset.
Methods: review of all pediatric transplants done for metabolic liver disease at our center over the recent 20 year cohort.
Results: Thirty one patients received LT for metabolic defects. Median age was 4.4 years, 24 patients received whole livers and 7 patients  partial livers. Indications are shown in tabl

Familial Cholestasis 6
Glycogen Storage disorders 1
Carbamoyl phosphate syndrome 1
Maple syrup 1
Propionic acidemia 1
Citrullinemia 1
Primary Oxaluria 1
Methylmalonic acidemia 1
Wilsons 2
Alpha 1 antitrypsin 8
Criggler najjar 1
Walcott Rallison syndrome 1

The patient and graft survival were compared to 131 transplants performed during the same era for other liver diseases.

Our findings confirm that metabolic liver disease is a highly successful indication for liver transplantation with exceptional outcomes. The long term survival for children we report demonstrates a realistic expectation of close to normal life potential.