These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: Perioperative measures in very long chain acyl-CoA dehydrogenase deficiency.
    Author: Vellekoop P, Diekman EF, van Tuijl I, de Vries MM, van Hasselt PM, Visser G.
    Journal: Mol Genet Metab; 2011 May; 103(1):96-7. PubMed ID: 21333574.
    Abstract:
    Surgical procedures in patients with metabolic disorders require specific anesthetic measures based on the nature of the involved metabolic disorder. Illustrated by the history of two patients, the need for a specific perioperative regimen in patients with very long chain acyl-CoA dehydrogenase deficiency (VLCADD) is discussed. One patient deteriorated, the other patient did well without any specific measurements. Although perioperative metabolic decompensation can currently not be predicted, it is a severe complication which should be avoided. We therefore advise to consider certain perioperative precautions in all VLCADD patients: 1) age and weight adapted glucose infusion, 2) stress avoiding premedication, 3) avoidance of volatile anesthetics, 4) avoidance of long chain fatty acid containing anesthetics and 5) perioperative glucose and CK monitoring.
    [Abstract] [Full Text] [Related] [New Search]