Review Question - QID 109597

A 3-week-old male is brought to the emergency department because of increasing lethargy. He was born at home without prenatal care or neonatal screening and appeared to be normal at birth. Despite this, his parents noticed that he would vomit after breastfeeding. He then progressively became more lethargic and began to have a few episodes of diarrhea after feeding. His parents do not recall any significant family history and neither of his siblings have had similar symptoms. Upon presentation, the infant is found to be generally unresponsive with mild hepatomegaly. Physical exam further reveals signs of clouding in the lenses of his eyes bilaterally. The levels of which of the following metabolites will be most dramatically elevated in this patient?