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
| OTC | 6 |
| 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.