title: Perioperative measures in very long chain acyl-CoA dehydrogenase deficiency.
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.
authors.affiliation: Department of Metabolic and Endocrine diseases, Wilhelmina Children's Hospital, UMC Utrecht, The Netherlands.
authors.forename: P
authors.initials: P
authors.lastname: Vellekoop
authors.forename: E F
authors.initials: EF
authors.lastname: Diekman
authors.forename: I
authors.initials: I
authors.lastname: van Tuijl
authors.forename: M M C
authors.initials: MM
authors.lastname: de Vries
authors.forename: P M
authors.initials: PM
authors.lastname: van Hasselt
authors.forename: G
authors.initials: G
authors.lastname: Visser
mesh_terms: D044942:Acyl-CoA Dehydrogenase, Long-Chain / Q000172:deficiency / Q000235:genetics; D000080984:Congenital Bone Marrow Failure Syndromes; D005260:Female; D005347:Fibroblasts / Q000201:enzymology; D006720:Homozygote; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008052:Lipid Metabolism, Inborn Errors; D008214:Lymphocytes / Q000201:enzymology; D008297:Male; D008661:Metabolism, Inborn Errors / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D028361:Mitochondrial Diseases / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D009135:Muscular Diseases / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D009154:Mutation / Q000235:genetics; D059035:Perioperative Period; D016896:Treatment Outcome
chemical_list: D044942:Acyl-CoA Dehydrogenase, Long-Chain