A patient presents with low blood levels of glucose and ketones in plasma during fasting, accompanied by a high level of plasma free fatty acids.A urine analysis reveals high levels of dicarboxylic acids.The patient is most likely to benefit from:

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Quizplus · Accepted Answer

This is a defect in b-oxidation.The high dicarboxylic acid levels are suggestive of medium-chain acyl-CoA dehydrogenase deficiency, rather than a defect of the carnitine shuttle.This condition leads to fasting hypoglycemia because gluconeogenesis requires adenosine triphosphate (ATP) from fatty acid oxidation during fasting, both for its energy requirement and as an activator of pyruvate carboxylase.

A Patient Presents with Low Blood Levels of Glucose and Ketones