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USMLE · Question #561

A 6-day-old breast-fed boy is brought to the emergency department by his mother because of poor weight gain and irritability since delivery, and a 2-hour history of vomiting. Physical examination show

The correct answer is B. Galactose 1-phosphate. See the full explanation below for the reasoning.

Question

A 6-day-old breast-fed boy is brought to the emergency department by his mother because of poor weight gain and irritability since delivery, and a 2-hour history of vomiting. Physical examination shows jaundice and hepatomegaly. A reducing substance test result of the urine is positive, and a glucose oxidase test result is negative. The concentration of which of the following metabolites in liver is most likely increased in this patient?

Options

  • AFructose 1, 6-bisphosphate
  • BGalactose 1-phosphate
  • CGlucose 1-phosphate
  • DGlucose 6-phosphate

How the community answered

(23 responses)
  • A
    9% (2)
  • B
    83% (19)
  • C
    4% (1)
  • D
    4% (1)

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