A 6-month-old Hispanic female infant begins having trembling with irritability (jittery spells) and vomiting after certain meals including baby food containing fruits. She showed a falloff in growth when pureed fruits and vegetables were introduced at age 4 months. She is hospitalized for evaluation, and blood obtained for testing when she exhibits tremors and irritability after a typical feeding. Serum glucose is 40 g/dL (normal 60-100) and plasma lactate 35 g/dL (normal 5-20), causing her physician to suspect hereditary fructose intolerance (a deficiency of the enzyme aldolase B). The symptoms and serum abnormalities of this disease are due to which of the following?
A. Accumulation of hexose phosphates, phosphate and ATP depletion, defective electron transport, and glycogen phosphorylase inhibition
B. Accumulation of triose phosphates, phosphate and ATP excess, defective glycolysis, and glycogen synthase inhibition
C. Accumulation of hexose phosphates, phosphate and ATP excess, defective electron transport, and glycogen phosphorylase stimulation
Please explain why the correct answer is right and what makes the others wrong.
A 6-month-old Hispanic female infant begins having trembling with irritability (jittery spells) and vomiting after certain meals including baby food containing fruits. She showed a falloff in growth when pureed fruits and vegetables were introduced at age 4 months. She is hospitalized for evaluation, and blood obtained for testing when she exhibits tremors and irritability after a typical feeding. Serum glucose is 40 g/dL (normal 60-100) and plasma lactate 35 g/dL (normal 5-20), causing her physician to suspect hereditary fructose intolerance (a deficiency of the enzyme aldolase B). The symptoms and serum abnormalities of this disease are due to which of the following?
A. Accumulation of hexose phosphates, phosphate and ATP depletion, defective electron transport, and glycogen phosphorylase inhibition
B. Accumulation of triose phosphates, phosphate and ATP excess, defective glycolysis, and glycogen synthase inhibition
C. Accumulation of hexose phosphates, phosphate and ATP excess, defective electron transport, and glycogen phosphorylase stimulation
Please explain why the correct answer is right and what makes the others wrong.