A patient with glutaric aciduria type I requires careful management in the perioperative period.
Pathophysiology: deficiency in glutaryl-CoA dehydrogenase
Concerns:
(1) acute striatal injury (resulting in dystonia) following any catabolic stress
(2) acute encephalopathy triggered by surgery, fever or infection
Things to avoid:
(1) high protein load
(2) high dose vasopressor therapy (especially epinephrine)
(3) deliberate hyperventilation
(4) anything associated with systemic inflammatory response
(5) postoperative infection
The patient's diet should be planned in consultation with a metabolic disease expert. Up until the age of 6 years the patient requires a low-lysine diet with additional administration of lysine-free, tryptophan-reduced, arginine-containing amino acid mixture. Carnitine supplementation is also recommended.
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