A 5-Day-Old Term Newborn Is Brought to the Emergency Department

A 5-day-old term newborn is brought to the emergency department for multiple episodes of emesis.  The breastfed infant has been having fewer wet diapers over the last 2 days.  Vital signs show tachycardia, tachypnea, and hypotension.  Physical examination shows an icteric, lethargic baby with a sunken fontanelle, dry mucous membranes, and hepatomegaly.  A blood culture is drawn and empiric antibiotics are initiated.  Hematologic studies show leukocytosis with bandemia.  Serum studies show hypoglycemia and elevated transaminases.  The ammonia level is normal.  Newborn screen is pending.  Preliminary results from arterial blood culture show gram-negative rods.  The infant is placed on a special formula and gradually improves over the next few days.  Which of the following steps in metabolism is most likely impaired in this patient?

A) A
B) B
C) C
D) D
E) E

Correct Answer:

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