Liver Transplantation Outcomes in Crigler-Najjar Syndrome in Iran: A Single-Center Retrospective Cohort Study Over 20 Years.

BACKGROUND AND AIMS: Crigler-Najjar Syndrome (CNS) is a rare autosomal recessive disorder caused by uridine diphosphate glucuronosyltransferase (UGT1A1) deficiency, leading to unconjugated hyperbilirubinemia. Without treatment, patients are at high risk of kernicterus and irreversible neurological damage. Liver transplantation (LT) is the only curative option. This study aims to evaluate long-term outcomes of LT in CNS patients over a 20-year period in Iran.

METHODS

In this retrospective cohort study, we analyzed 69 patients with CNS who underwent LT between 2000 and 2023 at Namazi Hospital and Abu Ali Sina Transplant Center in Shiraz, Iran. Long-term follow-up data, graft function, patient survival, and pre- and post-LT outcomes were evaluated.

RESULTS

Posttransplantation, bilirubin metabolism and liver function normalized rapidly. Within the first year, the overall mortality rate was 14.5%, mainly due to graft failure and sepsis. Late mortality (2.9%) was predominantly linked to chronic rejection and infections.

CONCLUSIONS

LT remains a definitive curative option for patients with CNS. However, postoperative complications, particularly hematologic and renal dysfunction, remain significant concerns. These findings underscore the importance of ongoing monitoring and optimization of post-LT care. Furthermore, the lack of consensus regarding ideal timing for LT highlights the need for future prospective studies to better inform clinical decision-making.