Anesthetic Care of a Child With Propionic Acidemia

Mahmood Rafiq, Joseph D. Tobias


Propionic acidemia (PA), an autosomal recessive inborn error of metabolism, is one of the most frequent organic acidurias. The primary defect is deficiency of the mitochondrial enzyme, propionyl-CoA carboxylase which plays a key role in the final catabolism of the branched chain amino acids, the end-products of the beta-oxidation of fatty acids, and the metabolism of the cholesterol side chains. Derangements in these pathways result in the accumulation of toxic metabolites which result in progressive end-organ dysfunction. Clinical manifestations include episodes of severe metabolic ketoacidosis, intercurrent infection, vomiting, hypotonia, convulsions, developmental delay with central nervous system involvement, gastroesophageal reflux, osteoporosis, pancreatitis, and cardiomyopathy. We present a 14-year-old girl with PA who required anesthetic care for magnetic resonance imaging (MRI) of the thoracolumbar spine. Previous reports of anesthetic care for these patients are reviewed, the end-organ involvement is discussed, and options for anesthetic care are presented.

J Med Cases. 2015;6(9):420-425


Propionic acidemia; Organic acidemia; Mitochondrial disorder

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