Liver transplantation for pediatric inherited metabolic disorders: Considerations for indications, complications, and perioperative management

Kimihiko Oishi, Ronen Arnon, Melissa P. Wasserstein, George A. Diaz

Research output: Contribution to journalReview article

14 Scopus citations

Abstract

LT is an effective therapeutic option for a variety of IEM. This approach can significantly improve the quality of life of patients who suffer from severe disease manifestations and/or life-threatening metabolic decompensations despite medical/dietary management. Due to the significant risks for systemic complications from surgical stressors, careful perioperative management is vital. Even after LT, some disorders require long-term dietary restriction, medical management, and monitoring of metabolites. Successful liver transplant for these complex disorders can be achieved with disease- and patient-specific strategies using a multidisciplinary approach. In this article, we review indications, complications, perioperative management, and long-term follow-up recommendations for IEM that are treatable with LT.

Original languageEnglish (US)
Pages (from-to)756-769
Number of pages14
JournalPediatric Transplantation
Volume20
Issue number6
DOIs
Publication statusPublished - Sep 1 2016

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Keywords

  • inborn errors of metabolism
  • long-term follow-up
  • pediatric liver transplantation
  • perioperative management

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

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