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Organic acids are key metabolites of virtually all pathways of intermediary metabolism as well as exogenous compounds such as drugs and special diets. Urine organic acids can be used to diagnose disorders of amino acid, organic acid, fatty acid and carbohydrate metabolism, among others, and can also be used for monitoring known patients. In many disorders, urine organic acid analysis is normal while the patient is well. Testing while symptomatic is usually the most informative.
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