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Defects in fatty acid oxidation and how hypoglycemia occurs

With the deficiency of long and medium chain fatty acid coenzyme A dehydrogenase deficiency there is a common form of fasting hypoglycemia which is very severe associated with hepatomegaly cardiomyopathy and hypotonia

Plasma carnitine levels are low
ketones are not present

These patients present with  hypotonia seizures and acrid order

The final diagnosis is established from evaluation of enzyme activity in liver biopsy tissue or cultured fibroblasts

These patients must avoid fasting and their diets should be supplemented with carnitine.

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