Metabolic disorders and anesthesia Journal Article


Authors: Yeoh, C.; Teng, H.; Jackson, J.; Hingula, L.; Irie, T.; Legler, A.; Levine, C.; Chu, I.; Chai, C.; Tollinche, L.
Article Title: Metabolic disorders and anesthesia
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. © 2019, Springer Science+Business Media, LLC, part of Springer Nature.
Keywords: review; liver transplantation; pathophysiology; propofol; hematopoietic stem cell transplantation; hemochromatosis; benzodiazepine derivative; amino acid; disorders of carbohydrate metabolism; metabolic disorder; cyanocobalamin; carnitine; anesthesia; lysosome storage disease; wilson disease; protein restriction; disorders of mitochondrial functions; anesthetic agent; botulinum toxin; metabolic disorders; branched chain amino acid; amino acid metabolism; antisense therapy; bicarbonate; phlebotomy; hyperhomocysteinemia; glycogen storage disease; phenylketonuria; human; priority journal; carbohydrate metabolism; lysosomal storage disorders; branched-chain amino acid metabolism; metal metabolism disorders; mitochondrial disorders; organic acidemias; urea cycle disorders; acidemia; disorders of amino acid and protein metabolism; disorders of metal metabolism; galactosemia; maple syrup urine disease; urea cycle disorder
Journal Title: Current Anesthesiology Reports
Volume: 9
Issue: 3
ISSN: 2167-6275
Publisher: Springer  
Date Published: 2019-09-01
Start Page: 340
End Page: 359
Language: English
DOI: 10.1007/s40140-019-00345-w
PROVIDER: scopus
PMCID: PMC6690620
PUBMED: 31406490
DOI/URL:
Notes: Review -- Source: Scopus
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  1. Cindy Beng-Imm Yeoh
    35 Yeoh
  2. Howard   Teng
    8 Teng
  3. Takeshi Irie
    14 Irie
  4. Aron S Legler
    7 Legler