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Liver transplantation for pediatric inherited metabolic disorders: considerations for indications, complications, perioperative management #MMPMID27329540
Oishi K; Arnon R; Wasserstein MP; Diaz GA
Pediatr Transplant 2016[Sep]; 20 (6): 756-69 PMID27329540show ga
Liver transplantation is an effective therapeutic option for a variety of inborn errors of metabolism. 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 liver transplantation, 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 inborn errors of metabolism that are treatable with liver transplantation.