Abstract

Background. Crigler-Najjar syndrome (CNS) is a rare genetic disorder found in less than 1 per 1,000,000 births. It happens as a result of an error in UGT1A1 enzyme which can cause high unconjugated bilirubin levels. Objective. To describe liver histology changes in patients who have undergone liver transplantation. Methods. This retrospective cross-sectional study was performed to evaluate the liver pathologies of patients with type 1 of Crigler-Najjar syndrome (CNS1). We analyzed medical records and liver histologic specimens of 53 children who were transplanted in Namazi Hospital Organ Transplant Center affiliated with Shiraz University of Medical Sciences between 2009 and 2019. We studied the tissue of the explanted liver, which was replaced by transplants. Most of the patients were less than 2 years old, with an average age of 1.7 years. The collected data were analyzed using SPSS 22 software. Results. The prevalent pathology found in the liver of these patients was periportal fibrosis (96.2). Cholestasis was the second common finding (94.3) followed by pericentral fibrosis (86.7) and ductal reaction (22.6). A significant correlation was only present between phototherapy time and ductal reaction grade Conclusion. Our results indicated a high prevalence of fibrosis of different grades among CNS 1 patients which bolds the necessity of histologic examination before considering treatments such as gene therapy or hepatocyte transplantation. © M. ATAOLLAHI, S.M. DEHGHANI, M.H. ANBARDAR, P. SHAKORANI, I. SHAHRAMIAN, M. SALARZAEI, F. PAROOIE.