Anesthetic considerations in medium-chain acyl-CoA dehydrogenase deficiency

Lisa A Caplan; Mary (Toni) A Felberg

doi:10.22037/jcma.v2i2.15356

Journal of Cellular & Molecular Anesthesia

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https://doi.org/10.22037/jcma.v2i2.15356

Anesthetic considerations in medium-chain acyl-CoA dehydrogenase deficiency

Author(s):

Lisa A Caplan^1,*,

Mary (Toni) A Felberg²

1Assistant Professor of Anesthesiology Baylor College of Medicine Department of Anesthesiology, Perioperative and Pain Medicine Texas Children’s Hospital Houston, Texas

2Assistant Professor of Anesthesiology Baylor College of Medicine Department of Anesthesiology, Perioperative and Pain Medicine Texas Children’s Hospital Houston, Texas

Journal of Cellular & Molecular Anesthesia:Vol. 2, issue 2; e149536

Published online:May 12, 2017

Article type:Review

How to Cite:Lisa A CaplanMary (Toni) A FelbergAnesthetic considerations in medium-chain acyl-CoA dehydrogenase deficiency.J Cell Mol Anesth.2(2):e149536.https://doi.org/10.22037/jcma.v2i2.15356.

Abstract

In the 1980?s, medium-chain acyl-CoA dehydrogenase deficiency (MCADD) was first described in the literature as three children who presented with coma, hypoglycemia, hyperammonemia, and fatty liver while fasting. These symptoms while similar to Reye?s Syndrome, were found to be due to an inability to metabolize medium chain fatty acids during fasting periods. Fatty acids are utilized by the body asessential fuel for skeletal and cardiac muscle, and as an important source of energy during fasting periods. Medium-chain acyl-CoA dehydrogenase is a mitochondrial enzyme required for the beta oxidation of medium chain fatty acids (C4-14), which is deficient in this syndrome. Anesthesiologists may come across these patients in their practice, as MCADD is the most common inhertited disorder of mitochondrial fatty acid oxidation. In addition to determining NPO timing and IV fluid selection, other preoperative issues anesthesiologists must consider are medication management to avoid metabolic decompensation. This manuscript will consider a pediatric patient with MCADD who presented to our pediatric hospital and received a general anesthetic following the guidelines created by ourmultidisciplinary perioperative team.

Keywords

medium-chain acyl-CoA dehydrogenase deficiency

metabolic disorder

MCADD

References

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References are in the PDF file of the article.

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Author(s):

Lisa A Caplan:[PubMed][Scholar]
Mary (Toni) A Felberg:[PubMed][Scholar]