Which of the following symptoms can occur frequently in infants suffering from medium-chain acyl-CoA dehydrogenase (MCAD) deficiency if periods between meals are protracted?

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Hypoglycemia and metabolic acidosis with normal levels of ketones are frequent symptoms in infants with MCAD deficiency if periods between meals are protracted.  In infants, the supply of glycogen lasts less than 6 hours and gluconeogenesis is not sufficient to maintain adequate blood glucose levels. Normally, during periods of fasting (in particular during the night) the oxidation of fatty acids provides the necessary ATP to fuel hepatic gluconeogenesis as well as ketone bodies for nonhepatic tissue energy production. In patients with MCAD deficiency there is a drastically reduced capacity to oxidize fatty acids. This leads to an increase in glucose usage with concomitant hypoglycemia. The deficit in the energy production from fatty acid oxidation, necessary for the liver to use other carbon sources, such as glycerol and amino acids, for gluconeogenesis further exacerbates the hypoglycemia. Normally, hypoglycemia is accompanied by an increase in ketone formation from the increased oxidation of fatty acids. In MCAD deficiency there is a reduced level of fatty acid oxidation, hence near-normal levels of ketones are detected in the serum.

Which of the Following Symptoms Can Occur Frequently in Infants