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. 2019 Sep;9(3):340-359.
doi: 10.1007/s40140-019-00345-w. Epub 2019 Jul 12.

Metabolic Disorders and Anesthesia

Cindy Yeoh  1 , Howard Teng  1 , Jacob Jackson  1 , Lee Hingula  1 , Takeshi Irie  1 , Aron Legler  1 , Corrine Levine  2 , Iris Chu  2 , Casey Chai  2 , Luis Tollinche  1
Affiliations

Metabolic Disorders and Anesthesia

Cindy Yeoh et al. Curr Anesthesiol Rep. 2019 Sep.

Abstract

Purpose of review: Metabolic disorders encompass a group of inherited inborn errors of metabolism that are uncommonly encountered but can pose challenges when encountered during the perioperative period. Hence, it is paramount that anesthesiologists are experienced and familiar with management of these conditions.

Recent findings: Hundreds of inborn errors of metabolism have already been identified, yet new metabolic disorders continue to be discovered with advancements in genomic science.

Summary: In our general review, we define the more common metabolic disorders encountered in perioperative medicine and discuss the perioperative anesthetic considerations and challenges associated with each disorder. The following disorders are covered in our review: disorders of carbohydrate metabolism, disorders of amino acid metabolism, disorders of branched-chain amino acid metabolism, organic acidemias, mitochondrial disorders, lysosomal storage disorders, metal metabolism disorders, and urea cycle disorders.

Keywords: Amino acid metabolism; Anesthesia; Branched-chain amino acid metabolism; Carbohydrate metabolism; Lysosomal storage disorders; Metabolic disorders; Metal metabolism disorders; Mitochondrial disorders; Organic acidemias; Urea cycle disorders.

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Conflict of interest statement

Conflict of Interest Cindy B. Yeoh, Howard C. Teng, Jacob C. Jackson, Lee P. Hingula, Takeshi Irie, Aron Legler, Corrine E. Levine, Iris Chu, Casey M. Chai, and Luis E. Tollinche declare they have no conflict of interest.

Figures

Fig. 1
Fig. 1
Biochemical pathway of the creation of homocysteine and specific enzymes/vitamins necessary for conversion to methionine vs. cystathionine [27]
Fig. 2
Fig. 2
Metabolic pathway. Defect in A causes PA. Defect in B causes MMA
See this image and copyright information in PMC

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