E71.111
3-methylglutaconic aciduria
Approximate Synonyms
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Aciduria, 3 methylgluaconic
Clinical Information
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A group of five inherited disorders caused by mutations in the auh, dnajc19, opa3, and taz genes. The disorders are characterized by impairment in the function of mitochondria, resulting in the accumulation and excretion of 3-methylglutaconic acid, and the presence of 3-methylglutaric acid in the urine.
Details
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E71.111 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
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The 2023 edition of ICD-10-CM E71.111 became effective on October 1, 2022.
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This is the American ICD-10-CM version of E71.111 – other international versions of ICD-10 E71.111 may differ.