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Review Question - QID 211024

Amino Acid Catabolism Overview Urea cycle QID: 211024 (M1.OMB.18.1)
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QID 211024 (Type "211024" in App Search)
A 2-day-old male infant is brought to the emergency department by ambulance after his parents noticed that he was convulsing and unresponsive. He was born at home and appeared well initially; however, within 24 hours he became increasingly irritable and lethargic. Furthermore, he stopped feeding and began to experience worsening tachypnea. This continued for about 6 hours, at which point his parents noticed the convulsions and called for an ambulance. Laboratories are obtained with the following results:

Orotic acid: 9.2 mmol/mol creatinine (normal: 1.4-5.3 mmol/mol creatinine)
Ammonia: 135 µmol/L (normal: < 50 µmol/L)
Citrulline: 2 µmol/L (normal: 10-45 µmol/L)

Which of the following treatments would most likely be beneficial to this patient?
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