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61912 Pediatrics

The parents of a 2-week-old infant bring him to the emergency center in your local hospital for emesis and listlessness. Both of his parents wanted a natural birth, so he was born at home and has not yet been to see a physician. On examination, you find a dehydrated, listless, and irritable infant. Although you do not have a birth weight, the parents do not feel that he has gained much weight. He has significant jaundice. His abdominal examination is significant for both hepatomegaly and splenomegaly. Laboratory values include a total bilirubin of 15.8 mg/dL and a direct bilirubin of 5.5 mg/dL. His liver function tests are elevated and his serum glucose is 38 mg/dL. His admit urinalysis is negative for glucose but positive for Gram-negative rods; his urine and his blood ultimately grow E coll. Which of the following nutritional considerations should be considered in this child?

AAdministration of high doses of vitamin B6
BInitial diet free of branched-chain amino acids
CLactose free formula
DProtein restriction and supplementation with citrulline
Pediatrics
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