Outcome of liver transplantation and prevalence of liver fibrosis in Crigler‐Najjar syndrome
نویسندگان
چکیده
Abstract Introduction Crigler‐Najjar syndrome (CNS) is a rare inherited disorder that characterized by high levels of non‐hemolytic, unconjugated hyperbilirubinemia leading to brain damage and even death. Liver transplantation (LT) can correct the metabolic defect, but there are little data regarding LT in this patient cohort. The liver parenchyma has been considered be structurally normal CNS, growing evidence clinically silent histologically significant fibrosis CNS patients. Patients methods We included 13 patients our retrospective study who underwent at center. Patient survival, graft function, long‐term complications were evaluated over median follow‐up period 10 years (range: 1–16 years). In addition, prevalence relevant was characterized. Results overall survival among 100%. only 61.5%. During period, 5 had undergo retransplantation. More than 45% showed histological signs fibrosis. Conclusion remains definite therapeutic option for severe needs thoroughly clinical risk‐benefit‐ratio impact on quality life. Furthermore, hepatic parenchymal injury while evaluating future options CNS.
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ژورنال
عنوان ژورنال: Clinical transplantation
سال: 2021
ISSN: ['1399-0012', '0902-0063']
DOI: https://doi.org/10.1111/ctr.14219