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Biochemistry
An eight-month-old female infant presented with recurrent episodes of hypoglycemia, especially if time interval of feeding is increased. Dicarboxylic acid is present in the urine. Urine ketone bodies is negative. The child responded well to IV Glucose, less fat and more carbohydrate diet, frequent feeding. The child was diagnosed to be MCAD deficiency. What is the reason for hypoglycemia?
Increased dicarboxylic acid inhibit glycogenolysis
Lack of ATP to support gluconeogenesis
Lack of acetyl-CoA to favour glycogenolysis
Glycogen stores are inadequate in infants
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