Question

1. A 3-month-old boy presents with poor feeding and growth, low muscle tone (hypotonia), elevation of blood lactic acid (lactic acidemia), and mild acidosis (blood pH 7.3-7.35). The ratio of pyruvate to lactate in serum is elevated, and there is decreased conversion of pyruvate to acetyl coenzyme A in fibroblasts. Which of the following compounds should be considered for therapy? A. Thiamine B. Pyridoxine C. Free fatty acids D. Biotin E. Ascorbic acid 2. Which one of following statements about glycogen synthesis and utilization is CORRECT? A Liver synthesizes more glycogen when the hepatic portal blood concentration of glucose is high because of the activity of glucokinase in the liver. B. Glycogen reserves in liver and muscle will meet energy requirements for several days in prolonged fasting. C. Glycogen is synthesized in the liver in the fed state, then exported to other tissues in low-density lipoproteins. D. Muscle syntheses glycogen in the fed state because glycogen phosphorylase is activated in response to insulin. The plasma concentration of glycogen increases in the fed state.

          1. A 3-month-old boy presents with poor feeding and growth, low muscle tone (hypotonia), elevation
of blood lactic acid (lactic acidemia), and mild acidosis (blood pH 7.3-7.35). The ratio of pyruvate to
lactate in serum is elevated, and there is decreased conversion of pyruvate to acetyl coenzyme A in
fibroblasts. Which of the following compounds should be considered for therapy?
A. Thiamine B. Pyridoxine C. Free fatty acids D. Biotin E. Ascorbic acid
2. Which one of following statements about glycogen synthesis and utilization is CORRECT?
A Liver synthesizes more glycogen when the hepatic portal blood concentration of glucose is high
because of the activity of glucokinase in the liver.
B. Glycogen reserves in liver and muscle will meet energy requirements for several days in prolonged
fasting.
C. Glycogen is synthesized in the liver in the fed state, then exported to other tissues in low-density
lipoproteins.
D. Muscle syntheses glycogen in the fed state because glycogen phosphorylase is activated in
response to insulin. The plasma concentration of glycogen increases in the fed state.

1. A 3-month-old boy presents with poor feeding and growth, low muscle tone (hypotonia), elevation
of blood lactic acid (lactic acidemia), and mild acidosis (blood pH 7.3-7.35). The ratio of pyruvate to
lactate in serum is elevated, and there is decreased conversion of pyruvate to acetyl coenzyme A in
fibroblasts. Which of the following compounds should be considered for therapy?
A. Thiamine B. Pyridoxine C. Free fatty acids D. Biotin E. Ascorbic acid
2. Which one of following statements about glycogen synthesis and utilization is CORRECT?
A Liver synthesizes more glycogen when the hepatic portal blood concentration of glucose is high
because of the activity of glucokinase in the liver.
B. Glycogen reserves in liver and muscle will meet energy requirements for several days in prolonged
fasting.
C. Glycogen is synthesized in the liver in the fed state, then exported to other tissues in low-density
lipoproteins.
D. Muscle syntheses glycogen in the fed state because glycogen phosphorylase is activated in
response to insulin. The plasma concentration of glycogen increases in the fed state.

Added by Aaron Z.

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