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 | |
| State | Published - 2021 |
UN SDGs
This output contributes to the following UN Sustainable Development Goals (SDGs)
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SDG 3 Good Health and Well-being
Keywords
- pediatric anesthesia
- pediatric liver transplantation
- pediatric metabolic disorder
- propionic acidemia
ASJC Scopus subject areas
- Epidemiology
- Safety, Risk, Reliability and Quality
- Safety Research
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