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

Paediatric Anaesthesia

Volumn 12, Issue 2, 2002, Pages 187-191

  Frei, F ^a   Studer, W ^a   Steiner, L ^a   Baumgartner, E ^a  

^a UNIVERSITY CHILDREN S HOSPITAL   (Switzerland)

Author keywords

Anaesthesia; Child; Fatty acids; Inborn errors; Lipid metabolism; Propofol

Indexed keywords

ACYL COENZYME A DEHYDROGENASE; ALFENTANIL; BUPIVACAINE; LIDOCAINE; MIDAZOLAM; NALBUPHINE; THIOPENTAL; VERY LONG CHAIN FATTY ACID;

ARTICLE; CASE REPORT; CIRCUMCISION; CLINICAL FEATURE; ELECTROLYTE INTAKE; ENZYME DEFICIENCY; FATTY ACID METABOLISM; GENERAL ANESTHESIA; GLUCOSE INFUSION; HUMAN; INBORN ERROR OF METABOLISM; MALE; PERIOPERATIVE PERIOD; POSTOPERATIVE ANALGESIA; PRIORITY JOURNAL; SCHOOL CHILD; STRESS; SURGICAL TECHNIQUE; TREATMENT OUTCOME;

ANESTHESIA, CONDUCTION; CHILD; CIRCUMCISION, MALE; FATTY ACID DESATURASES; FATTY ACIDS; HUMANS; INTRAOPERATIVE CARE; MALE; METABOLISM, INBORN ERRORS; PHIMOSIS; PREOPERATIVE CARE; STRESS, PSYCHOLOGICAL;

EID: 0036173759     PISSN: 11555645     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1460-9592.2002.00809.x     Document Type: Article

References (13)

1. Fatty acid mitochondrial beta-oxidation and hypoglycaemia in children
2. A novel disease with deficiency of mitochondrial very-long-chain acyl-CoA dehydrogenase
3. Very long chain acyl-CoA dehydrogenase deficiency: Identification of a new inborn error of mitochondrial fatty acid oxidation in fibroblasts
4. Mitochondrial very-long-chain acyl-coenzyme A dehydrogenase deficiency: Clinical characteristics and diagnostic considerations in 30 patients
5. Adolescent myopathic presentation in two sisters with very long-chain acyl-CoA dehydrogenase deficiency
6. Very long chain acyl-coenzyme A dehydrogenase deficiency with adult onset
7. Death caused by perioperative fasting and sedation in a child with unrecognized very long chain acyl-coenzyme A dehydrogenase deficiency
8. Preoperative increase in the plasma concentration of free fatty acids during minor elective interventions using a conventional anesthesia technique with enflurane
9. Effects of glucose and fatty acids on myocardial ischaemia and arrhythmias
10. Impaired fatty acid oxidation in propofol infusion syndrome
11. Reversal of severe hypertrophic cardiomyopathy and excellent neuropsychologic outcome in very-long-chain acyl-coenzyme A dehydrogenase deficiency
12. Prophylaxis of early ventricular fibrillation by inhibition of acylcarnitine accumulation
13. Acylcarnitine accumulation does not correlate with reperfusion recovery in palmitate-perfused rat hearts