NBME 2 blk 3 qs 6 - kooqi

[ArchivalUser]

A 2-month-old boy is brought to the physician because
of a 6-week
history of persistent diarrhea and vomiting, most
pronounced after formula
feedings. He has had a 113-g (4-oz) weight loss since
birth. He
currently weighs 3100 g (6 lb 13 oz) and is 51 cm (20
in) in length. He
appears irritable. Examination shows jaundice. The
lungs are clear to
auscultation. No murmurs are heard. The liver is
palpated 2 to 3 cm
below the right costal margin, and the spleen is
palpated 1 to 2 cm below
the left costal margin. Laboratory studies show:


Serum
Glucose 35 mg/dL
Bilirubin (total) 2.3 mg/dL
Urine
Glucose negative
Reducing substances 3+

Which of the following is the most likely mechanism of
these findings?

A
) Decreased gluconeogenesis

B
) Decreased insulin secretion

C
) Increased glucagon secretion

D
) Increased gluconeogenesis

E
) Increased insulin secretion



plz explain..thanx

[ArchivalUser]

In individuals with galactosemia, the enzymes needed for further metabolism of galactose are severely diminished or missing entirely, leading to toxic levels of galactose in the blood, resulting in hepatomegaly (an enlarged liver), cirrhosis, renal failure, cataracts, brain damage, and ovarian failure. Without treatment, mortality in infants with galactosemia is about 75%.

itsa galactosemia with liver failure leading to decreased gluconeogenesis and hypoglycemia . A