P079: Acute mitochondrial dysfunction in proprionic acidemia

In consideration of severe metabolic decompensation without a clear etiology and a normal ammonia, our treatment plan framework shifted towards consideration of mitochondrial dysfunction in a patient under significant physiologic stress. Due to concern for altered mental status head CT was done and was normal. Dextrose infusion was stopped and the patient was placed on a bicarbonate drip until his blood pH normalized. Nutritional support was given via TPN with limited dextrose. He was given a mitochondrial cocktail consisting of Co-Q10, thiamine, and N-acetylcysteine.