Anesthetic Management for Pediatric Liver Transplantation in a Patient With Propionic Acidemia: A Case Report

Luis I. Rodriguez; Erica J. Tainsh; Eliane Varga; Ana C. Mavarez

doi:10.1177/23247096211015025

Anesthetic Management for Pediatric Liver Transplantation in a Patient With Propionic Acidemia: A Case Report

Luis I. Rodriguez, Erica J. Tainsh, Eliane Varga, Ana C. Mavarez

Anesthesiology and Perioperative Medicine

Research output: Contribution to journal › Article › peer-review

1 Scopus citations

Abstract

Propionic acidemia is an inborn error of metabolism characterized by accumulation of propionic acid due to deficiency of propionyl-CoA carboxylase. Main stay of treatment focuses on reducing dietary protein. However, orthotropic liver transplantation decreases the frequency of metabolic decompensations and improves life expectancy. We report a case of a 4-year-old boy undergoing orthotropic liver transplantation to treat propionic acidemia. This case highlights the use of intraoperative monitoring of metabolic markers like urine ketones, arterial ammonia, and lactate levels as these patients are at risk for hyperammonemia and metabolic acidosis. Also, the relevance in outcomes when performing early extubation in fast-tracking recovery.

Original language	English (US)
Journal	Journal of Investigative Medicine High Impact Case Reports
Volume	9
DOIs	https://doi.org/10.1177/23247096211015025
State	Published - 2021

Keywords

pediatric anesthesia
pediatric liver transplantation
pediatric metabolic disorder
propionic acidemia

ASJC Scopus subject areas

Epidemiology
Safety, Risk, Reliability and Quality
Safety Research

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1177/23247096211015025

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abstract = "Propionic acidemia is an inborn error of metabolism characterized by accumulation of propionic acid due to deficiency of propionyl-CoA carboxylase. Main stay of treatment focuses on reducing dietary protein. However, orthotropic liver transplantation decreases the frequency of metabolic decompensations and improves life expectancy. We report a case of a 4-year-old boy undergoing orthotropic liver transplantation to treat propionic acidemia. This case highlights the use of intraoperative monitoring of metabolic markers like urine ketones, arterial ammonia, and lactate levels as these patients are at risk for hyperammonemia and metabolic acidosis. Also, the relevance in outcomes when performing early extubation in fast-tracking recovery.",

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journal = "Journal of Investigative Medicine High Impact Case Reports",

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AU - Tainsh, Erica J.

AU - Varga, Eliane

AU - Mavarez, Ana C.

PY - 2021

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AB - Propionic acidemia is an inborn error of metabolism characterized by accumulation of propionic acid due to deficiency of propionyl-CoA carboxylase. Main stay of treatment focuses on reducing dietary protein. However, orthotropic liver transplantation decreases the frequency of metabolic decompensations and improves life expectancy. We report a case of a 4-year-old boy undergoing orthotropic liver transplantation to treat propionic acidemia. This case highlights the use of intraoperative monitoring of metabolic markers like urine ketones, arterial ammonia, and lactate levels as these patients are at risk for hyperammonemia and metabolic acidosis. Also, the relevance in outcomes when performing early extubation in fast-tracking recovery.

KW - pediatric anesthesia

KW - pediatric liver transplantation

KW - pediatric metabolic disorder

KW - propionic acidemia

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Anesthetic Management for Pediatric Liver Transplantation in a Patient With Propionic Acidemia: A Case Report

Abstract

Keywords

ASJC Scopus subject areas

UN SDGs

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