Why does MCAD cause hypoglycemia?

When you don’t have enough of the MCAD enzyme in your body, certain fats called medium-chain fatty acids can’t be broken down and converted to energy. This results in hypoglycemia and low energy. Also, fatty acids can build up in body tissues and cause damage to the liver and brain.

Why does fasting exacerbate the symptoms of MCAD?

Individuals with MCADD experience symptoms of metabolic crisis due to low blood sugar (hypoglycemia) after periods of prolonged fasting or in response to a common illness. These may include weakness, vomiting, and seizures.

How do you know if you have MCAD?

MCAD deficiency is diagnosed through newborn screening followed by genetic testing. Newborn screening. In the U.S., all states screen for MCAD deficiency at birth. If screening levels are abnormal, additional testing can be done.

Why does impaired fatty acid oxidation cause hypoglycemia?

Hypoglycemia as one major clinical sign in all fatty acid oxidation defects occurs due to a reduced hepatic glucose output and an enhanced peripheral glucose uptake rather than to transcriptional changes that are also observed simultaneously as presented in medium-chain acyl-CoA dehydrogenase (MCAD) -deficient mice.

How common is medium chain acyl CoA dehydrogenase deficiency?

In the United States, the estimated incidence of MCAD deficiency is 1 in 17,000 people. The condition is more common in people of northern European ancestry than in other ethnic groups.

What is medium chain acyl CoA dehydrogenase deficiency?

Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is an inherited disorder that prevents your body from breaking down certain fats and converting them into energy.

How does MCAD deficiency affect your sugar level?

Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is an inherited disorder that prevents your body from breaking down certain fats and converting them into energy. As a result, the level of sugar in your blood can drop dangerously low (hypoglycemia). MCAD deficiency is present from birth and is a lifelong condition.

Is there a cure for medium chain acyl coenzyme A deficiency?

MCADD is caused by mutations in the ACADM gene and inheritance is autosomal recessive. [1] Treatment includes strict avoidance of fasting and avoidance of medium chain triglycerides in the diet. [2] If not treated, people with MCADD are at risk of serious complications including sudden death.

How does hypoglycemia cause symptoms of MCADD?

Hypoglycemia is the direct cause of the symptoms of MCADD. However, precursor molecules and metabolites that become “stuck” in the biochemical pathway of fat breakdown due to deficient MCAD can build up in the body and cause some oxidative damage. MCADD is an autosomal recessive genetic condition.