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

Full Text: HTML PDF

Browse  Journals  


Journal of clinical Medicine Research

Journal of Endocrinology and Metabolism

Journal of Clinical Gynecology and Obstetrics

World Journal of Oncology

Gastroenterology Research

Journal of Hematology

Journal of Medical Cases

Journal of Current Surgery

Clinical Infection and Immunity

Cardiology Research

World Journal of Nephrology and Urology

Cellular and Molecular Medicine Research

Journal of Neurology Research

International Journal of Clinical Pediatrics






Journal of Medical Cases, monthly, ISSN 1923-4155 (print), 1923-4163 (online), published by Elmer Press Inc.            
The content of this site is intended for health care professionals.
This is an open-access journal distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License, which permits unrestricted
non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Creative Commons Attribution license (Attribution-NonCommercial 4.0 International CC-BY-NC 4.0)

This journal follows the International Committee of Medical Journal Editors (ICMJE) recommendations for manuscripts submitted to biomedical journals,
the Committee on Publication Ethics (COPE) guidelines, and the Principles of Transparency and Best Practice in Scholarly Publishing.

website:   editorial contact:
Address: 9225 Leslie Street, Suite 201, Richmond Hill, Ontario, L4B 3H6, Canada

© Elmer Press Inc. All Rights Reserved.