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Liver Transplantation for Inherited Metabolic Disorders: A Work in Progress.

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Liver transplant (LT) for Inherited Metabolic Disorders (IMD) showed improving outcomes over 30 years. While survival rates are lower than global averages, recent data indicates progress in patient and graft survival for IMD patients.

Keywords:
hepatic artery thrombosisinherited metabolic disordersliver transplantationmetabolic liver diseaseorganic acidaemia

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Area of Science:

  • Hepatology
  • Transplantation Surgery
  • Genetics

Background:

  • Inherited Metabolic Disorders (IMD) are a significant indication for liver transplantation (LT).
  • Long-term outcomes of LT for IMD require continuous evaluation to refine treatment protocols.

Purpose of the Study:

  • To analyze trends in indications and outcomes of liver transplantation for IMD over three decades.
  • To assess changes in patient and graft survival rates for IMD patients undergoing LT.

Main Methods:

  • Retrospective review of 62 LTs for IMD between 1989 and 2021.
  • Collection of demographic, clinical data, indications, complications, and outcomes.
  • Kaplan-Meier analysis for patient and graft survival.

Main Results:

  • 55 patients underwent 62 LTs, with common complications including rejection and bile leak.
  • Mortality rates for IMD significantly decreased over the decades, from 53.3% (1989-2000) to 8.3% (2011-2021).
  • Cumulative 10-year patient survival was 74.5% and graft survival was 66.1%.

Conclusions:

  • Liver transplantation outcomes for IMD have improved over the last decade.
  • Enhanced patient and donor selection, alongside protocol modifications, contribute to better results.
  • While survival rates lag behind global reports, the trend indicates progress in managing IMD via LT.