Failure to Thrive and Short-term Survival of Liver Transplant Recipients with Tyrosinemia

Abstract

Abstract

Background: Liver transplantation is the main treatment for tyrosinemia. The pre-operative conditions such as the 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. The 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: The presence of FTT in patients with tyrosinemia undergoing liver transplantation, would significantly decrease the short-term mortality rate in transplant recipients.