Introduction: Hereditary tyrosinemia type 1 is a rare autosomal recessive metabolic disorder with an estimated incidence of 1/100,000 to 1/120,000 worldwide. We aimed to evaluate the outcome of liver transplantation in patients with tyrosinemia.

Methods: This study was conducted in the Shiraz transplant center, affiliated to Shiraz University of Medical Sciences, in Abu Ali hospital, Shiraz, Iran. All pediatric patients who underwent liver transplantation for tyrosinemia were included in this report.

Results: Overall 107 patients entered this study. The majority of patients were male (56%) with a median age of 36 months. Median (IQR) duration of liver disease was 19 months and median hospital stay was 2 days. Approximately, 14.9% of patients had cirrhosis in their first liver biopsy. Among tyrosinemic patients, 3 had HCC in histologic examination of the explanted liver. Median Pediatric end-stage liver disease (PELD) score at transplant was 13. Most of the participants had Child-Pugh class A (63.1%) with a mean score of 6.41 ± 1.9. Mean warm ischemic time and cold ischemic time was 45 and 80 minutes, respectively. Median follow-up duration was 54 months. During the study, 29 patients died which was mostly due to sepsis (34.5%). The overall 1-, 2-, 5-, and 15-year survival rates were 85%, 77.5%, 77.6% and 77.6%, respectively. The majority of our donors were living donors (51.4%).

Conclusion: This is the largest single-center report on pediatric liver transplantation and tyrosinemia.