Perioperative management of a child with very-long-chain acyl-coenzyme A dehydrogenase deficiency.

Détails

ID Serval
serval:BIB_79C5D304C575
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Titre
Perioperative management of a child with very-long-chain acyl-coenzyme A dehydrogenase deficiency.
Périodique
Paediatric Anaesthesia
Auteur(s)
Steiner L.A., Studer W., Baumgartner E.R., Frei F.J.
ISSN
1155-5645
Statut éditorial
Publié
Date de publication
2002
Peer-reviewed
Oui
Volume
12
Numéro
2
Pages
187-191
Langue
anglais
Notes
Publication types: Case Reports ; Journal Article
Publication Status: ppublish
Résumé
Very-long-chain acyl-coenzyme A dehydrogenase deficiency is an inborn error of fatty acid metabolism. The clinical presentation of this disease in children is either a severe form with onset of symptoms in the first months of life, cardiomyopathy, metabolic acidosis, myopathy and a high mortality, or a less severe form manifesting mainly with hypoglycaemia. Perioperative fasting and (even emotional) stress can trigger metabolic decompensation through the altered metabolism of endogenous fatty acids resulting in hypoglycaemia, acute cardiac and hepatic dysfunction and rhabdomyolysis. We report the perioperative management of a 9-year-old boy suffering from the severe form of this disease who underwent circumcision. Metabolism was kept stable in this child by using a glucose--electrolyte infusion throughout the perioperative period to avoid the biochemical consequences of fasting and a benzodiazepine--opioid technique combined with regional anaesthesia to minimize the stress response. Considering reports about a possible interference of propofol with fatty acid oxidation and to avoid the unnecessary administration of fatty acids, propofol should not be used in these patients.
Mots-clé
Anesthesia, Conduction, Child, Circumcision, Male, Fatty Acid Desaturases/deficiency, Fatty Acids/metabolism, Humans, Intraoperative Care, Male, Metabolism, Inborn Errors/metabolism, Phimosis/surgery, Preoperative Care, Stress, Psychological/prevention & control
Pubmed
Web of science
Création de la notice
29/12/2009 18:12
Dernière modification de la notice
03/03/2018 18:32
Données d'usage