title: Liver transplantation for pediatric inherited metabolic disorders: Considerations for indications, complications, and perioperative management.
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.
authors.affiliation: Departments of Pediatrics, Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
authors.forename: Kimihiko
authors.initials: K
authors.lastname: Oishi
authors.affiliation: Departments of Pediatrics, Pediatric Gastroenterology and Hepatology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.|The Recanati/Miller Transplantation Institute, Mount Sinai Medical Center, New York, NY, USA.
authors.forename: Ronen
authors.initials: R
authors.lastname: Arnon
authors.affiliation: Departments of Pediatrics, Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
authors.forename: Melissa P
authors.initials: MP
authors.lastname: Wasserstein
authors.affiliation: Departments of Pediatrics, Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
authors.forename: George A
authors.initials: GA
authors.lastname: Diaz
mesh_terms: D000359:Aftercare / Q000379:methods; D002648:Child; D006801:Humans; D016031:Liver Transplantation; D008661:Metabolism, Inborn Errors / Q000601:surgery*; D010372:Pediatrics; D019990:Perioperative Care / Q000379:methods; D011183:Postoperative Complications / Q000175:diagnosis / Q000628:therapy
keywords: inborn errors of metabolism; long-term follow-up; pediatric liver transplantation; perioperative management