*Purpose: Pediatric liver transplantation (LT) is an established treatment for a myriad of liver diseases most notably biliary atresia and urea cycle defects. In the past decade however, LT became an attractive treatment modality for other metabolic conditions. The purpose of this study is to describe LT experience at a single tertiary referral center for genetic and metabolic liver disease and report clinical outcomes including patient and graft survivals.

*Methods: We identified 91 patients were underwent LT for genetic and metabolic conditions in the past decade. Metabolic conditions included glycogen storage disease, maple syrup urine disease, and tyrosinemia. Genetic disorders included alpha-one anti-trypsin deficiency and Wilson’s disease. Multi-organ transplant patients were excluded. We reviewed baseline demographic data and clinical outcomes.

*Results: Patients with genetic and metabolic conditions demonstrate a positive trend for graft and patient survival when compared with other LT patients who do not have any genetic or metabolic disease (Fig 1). While it did not reach statistical significance, this patient population seem to have better outcomes, particularly five years post LT.

*Conclusions: There is a shift in LT treatment paradigm from lifesaving to improving quality of life and reducing detrimental long-term sequela of genetic and metabolic liver disease. At our institution, there is a trend in improved survival for patients with underlying genetic and metabolic conditions who undergo LT. More research is required to determine optimal timing for transplant intervention.

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