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content:acycarnitines [2020/02/16 17:56] – [Reference values] icna | content:acycarnitines [2020/02/16 17:59] – icna | ||
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suspected fatty acid oxidation disorders, acute myopathy, rhabdomyolysis, | suspected fatty acid oxidation disorders, acute myopathy, rhabdomyolysis, | ||
==== Notes ==== | ==== Notes ==== | ||
- | * Diagnostic | + | * diagnostic |
* serum acylcarnitines are useful in the diagnosis of fatty acid oxidation disorders and several organic acidurias | * serum acylcarnitines are useful in the diagnosis of fatty acid oxidation disorders and several organic acidurias | ||
* serum acylcarnitines also useful in evaluating treatment during follow-up of patients with fatty acid beta-oxidation disorders and several organic acidurias | * serum acylcarnitines also useful in evaluating treatment during follow-up of patients with fatty acid beta-oxidation disorders and several organic acidurias | ||
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==== Pathophysiology ==== | ==== Pathophysiology ==== | ||
- | Fatty acid oxidation (FAO) plays a major role in energy production during periods of fasting. When the body's supply of glucose is depleted, fatty acids are mobilized from adipose tissue, taken up by the liver and muscles, and oxidized to acetyl-CoA. In the liver, acetyl-CoA is the building block for the synthesis of ketone bodies, which enter the blood stream and provide an alternative substrate for production of energy in other tissues when the supply of glucose is insufficient to maintain a normal level of energy. The acyl groups are conjugated with carnitine to form acylcarnitines, | + | Fatty acid oxidation (FAO) plays a major role in energy production during periods of fasting. When the body's supply of glucose is depleted, fatty acids are mobilized from adipose tissue, taken up by the liver and muscles, and oxidized to acetyl-CoA. In the liver, acetyl-CoA is the building block for the synthesis of ketone bodies, which enter the blood stream and provide an alternative substrate for production of energy in other tissues when the supply of glucose is insufficient to maintain a normal level of energy. The acyl groups are conjugated with carnitine to form acylcarnitines, |
==== Reference values ==== | ==== Reference values ==== |