Abstract

Background: Liver transplantation is the main treatment for tyrosinemia. The pre-operative conditions such as presence of failure to thrive (FTT) would affect the prognosis of transplant recipients. Objective: To test whether the survival rate of liver transplant recipients with tyrosinemia was associated with pre-operative presence or absence of FTT. Methods: In a historical cohort study, the survival rate of 42 liver transplant recipients with FTT was compared with that of 68 patients without FTT during 6 months of the transplantation. Kaplan-Meier survival analysis and Cox regression were used for data analysis. Results: The recipients with and without FTT were matched for age, sex, baseline laboratory test results, and the frequency distribution of the underlying diseases. 35 (83) of 42 recipients with FTT and 29 (43) of 68 without FTT had rickets (p<0.001). Histopathological study of the resected livers revealed that 50 of those with FTT and 77 of recipients without FTT had hepatocellular carcinoma or hepatoblastoma (p=0.015). Recipients with FTT were followed for a median (IQR) of 62 (23–99) days; those without FTT, 31 (6–85) days. During the follow-up period, 9 (21) of 42 recipients with FTT and 32 (47) of 68 without FTT died. Recipients with FTT had a significantly (p=0.013) better survival. Presence of FTT in recipients was associated with a 65 reduction (HR 0.35, 95 CI 0.158–0.776) in the risk of death within 6 months of the transplantation. Conclusion: Presence of FTT in patients with tyrosinemia undergoing liver transplantation, would significantly decrease the short-term mortality rate in transplant recipients. © (2023), (Iranian Society for Organ Transplantation). All Rights Reserved.