1/2. Perioperative management of a child with very-long-chain acyl-coenzyme a dehydrogenase deficiency.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.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/2. Anaesthesia and pyruvate dehydrogenase deficiency.A ten-month-old infant with pyruvate dehydrogenase deficiency received anaesthesia on two occasions, once for a laparotomy and once for a tracheostomy. During both anaesthetics (different techniques) she developed an increase in arterial lactate levels and a metabolic acidosis. Pyruvate dehydrogenase deficiency results in the inability to metabolize pyruvate with resultant accumulation of pyruvate and lactate. Inhibition of gluconeogenesis, which may be produced by halothane and thiopentone, will also increase lactate levels. Other causes of increased lactate levels are hypocarbia and high carbohydrate intake. In this patient hypocarbia may have produced increased lactate levels and increased the metabolic acidosis. Recommendations include avoidance of halogenated anaesthetics, avoidance of lactate containing solutions, maintenance of normocarbia, and stress-free anaesthesia.- - - - - - - - - - ranking = 2keywords = anaesthesia (Clic here for more details about this article) |