Isovaleric Acidemia News and Information
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Latest Isovaleric Acidemia News
Isovaleric acidemia is a rare hereditary metabolic disorder in which the body is unable to process certain proteins properly. It is classified as an organic acid disorder, which is a condition that leads to an abnormal buildup of particular acids known as organic acids. The condition is characterized by a deficiency of the enzyme isovaleryl CoA dehydrogenase. Isovaleric acidemia occurs in both an acute and a chronic intermittent form. In the acute form of Isovaleric Acidemia, vomiting, refusal to eat, and listlessness usually occur Abnormal levels of organic acids in the blood (organic acidemia), urine (organic aciduria), and tissues can be toxic and can cause serious health problems.
With treatment and low protein diet, the disorder becomes chronically intermittent, and a nearly normal life is possible.