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Anesthetic Management of Patients With Inborn Errors of Metabolism

Kloesel, Benjamin MD, MSBS*; Holzman, Robert S. MD, MA (Hon), FAAP

doi: 10.1213/ANE.0000000000001689
Patient Safety: Narrative Review Article
Continuing Medical Education

Inborn errors of metabolism (IEM) are characterized by the body’s inability to convert food into energy. The pathogenetic mechanism is based on defects in a variety of cellular enzymes. In addition to impairment of energy generation, accumulation of substrates may occur, which can deposit in tissue and lead to organ dysfunction. IEM can have profound implications for perioperative management, including difficult airway management, cardiac dysfunction, aspiration risk, seizures, and metabolic dysregulation. For the anesthesiologist, comprehensive knowledge is difficult to attain because of the heterogeneity of this group and the low prevalence of specific diseases. The first part of this article reviews intermediary metabolism, whereas the second part aims to highlight important aspects in perioperative management of patients with IEM. Instead of reviewing each single disorder within the vast group of IEM, we provide a conceptual framework that will facilitate the understanding of main problems encountered in each of the disease subgroups.

Published ahead of print December 15, 2016.

From the *Department of Anesthesiology, University of Minnesota, Masonic Children’s Hospital, Minneapolis, Minnesota; and Department of Anesthesia, Perioperative and Pain Medicine, Children’s Hospital Boston, Boston, Massachusetts.

Published ahead of print December 15, 2016.

Accepted for publication September 26, 2016.

Funding: Departmental.

The authors declare no conflicts of interest.

Address correspondence to Benjamin Kloesel, MD, MSBS, Department of Anesthesiology, University of Minnesota, Masonic Children’s Hospital, B515 Mayo Bldg, 420 Delaware St SE, Minneapolis, MN 55455. Address e-mail to

© 2017 International Anesthesia Research Society
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