Step II aminoacidemia

[Fungi121]

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I was just doing a practice q that basically gave you a kid with maple syrup urine disease and asked what is something you could do to acutely manage him; he had low bicarb, low serum pH, high serum ammonia, low glucose;

answer was give him sodium benzoate---but this confuses me because this would cause you to lose ammonia = less base = more acidic = makes the acidosis worse?

Can someone help me out?

 

[UMN]

Sodium Benzoate binds to leucine, isoleucine, valine and other AA and impairs their absorption and increases their excretion. This results in decreased ammonia PRODUCTION. And obviously since it decreases the absorption of these AA, it will decrease the production of ketoacids.