Small group 1 - problem 4 Flashcards
Another infant with pyruvate carboxylase deficiency was treated with high doses of citrate, aspartate, and continuous drip feedings. Lactate and ketones diminished dramatically, but the child had profound mental retardation.
1
Q
Why would the child be treated with high doses of citrate and aspartate?
A
Because of the deficiency of pyruvate carboxylase, there is a decrease in OAA, an intermediate of the TCA cycle and a gluconeogenic precursor. Citrate and aspartate were added as precursors of oxaloacetate.
