Liver transplantation for pediatric inherited metabolic disorders: Considerations for indications,complications, and perioperative management |
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| Authors: | Kimihiko Oishi Ronen Arnon Melissa P. Wasserstein George A. Diaz |
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| Affiliation: | 1. Departments of Pediatrics, Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY, USA;2. Departments of Pediatrics, Pediatric Gastroenterology and Hepatology, Icahn School of Medicine at Mount Sinai, New York, NY, USA;3. The Recanati/Miller Transplantation Institute, Mount Sinai Medical Center, New York, NY, USA |
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| 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. |
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| Keywords: | pediatric liver transplantation inborn errors of metabolism long‐term follow‐up perioperative management |
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