• Pediatric transplantation · Sep 2016

    Review

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

    • Kimihiko Oishi, Ronen Arnon, Melissa P Wasserstein, and George A Diaz.
    • Departments of Pediatrics, Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
    • Pediatr Transplant. 2016 Sep 1; 20 (6): 756-69.

    AbstractLT 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. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

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