Liver Transplantation in a Patient with Crigler-Najjar Syndrome Type 1: A Case Report of Two Cases

Ahmed Uslu; Nedim Çekmen; Zeynep Ersoy; Adnan Torgay

doi:10.18502/aacc.v9i1.11953

Ahmed Uslu Başkent University, Anesthesiology and Reanimation, Ankara Hospital, Başkent University, Ankara, Turkey.
Nedim Çekmen Başkent University, Anesthesiology and Reanimation, Ankara Hospital, Başkent University, Ankara, Turkey.
Zeynep Ersoy Başkent University, Anesthesiology and Reanimation, Ankara Hospital, Başkent University, Ankara, Turkey.
Adnan Torgay Başkent University, Anesthesiology and Reanimation, Ankara Hospital, Başkent University, Ankara, Turkey.

DOI: https://doi.org/10.18502/aacc.v9i1.11953

Keywords: Unconjugated hyperbilirubinemia; Crigler najjar syndrome; Orthotopic liver transplantation; Phototherapy; Plasmapheresis

Abstract

Crigler Najjar syndrome(CNS); is a disease in which the diphosphate glucuronosyltransferase (bilirubin-UGT) enzyme function, which plays a role in the glucuronidation of bilirubin, is deficient as a result of mutation in the uridine 5'-diphosphate-glucuronosyltransferase 1A1 (UGT1A1) gene.1 As a result, non-hemolytic unconjugated hyperbilirubinemia is seen. Orthotopic liver transplantation (OLT) is seen as a curative treatment option in Crigler Najjar syndrome type 1 (CNS1). In this case report, we present our patients who were 11 months old and 8 years old with a diagnosis of CNS1, whose bilirubin levels were controlled by preoperative daily phototherapy and plasmapheresis, and who had OLT from their parents to two siblings. We wanted to show the importance of a close follow-up and multidisciplinary treatment approach in the early period before OLT in CNS1 patients and thus the benefit to the patient's prognosis in the postoperative period.