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Perioperative management of a child with very-long-chain acyl-coenzyme A dehydrogenase deficiency (2002)

STEINER, L.A. ; STUDER, W. ; BAUMGARTNER, E.R. ; [et al.]

Oxford UK : Blackwell Science Ltd.

Pediatric anesthesia 12 (2002), S. 0

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ISSN: 1460-9592

Source: Blackwell Publishing Journal Backfiles 1879-2005

Topics: Medicine

Notes: 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.

Type of Medium: Electronic Resource

URL: http://dx.doi.org/10.1046/j.1460-9592.2002.00809.x

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Amsterdam : Elsevier

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ISSN: 0959-440X

Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Topics: Biology , Medicine