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    v1.0.28
    OpenPayerBilling CodesMetabolic disordersICD-10-CM e71-disorders-of-branched-chain-amino-acid-metabolism
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    ICD-10-CM Code For Disorders Of Branched Chain Amino Acid Metabolism And Fatty Acid Metabolism: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group E71 includes disorders of branched-chain amino-acid metabolism and fatty-acid metabolism, such as maple syrup urine disease, multiple acyl-CoA dehydrogenase deficiency, and disorders of fatty-acid oxidation that impair energy production. These ICD-10-CM diagnosis codes are used to document metabolic crises, chronic metabolic dysfunction, organ system involvement, and the clinical complexity of hereditary or acquired metabolic enzyme deficiencies. Accurate coding within the ICD-10-CM E71 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM E71: Disorders Of Branched-Chain Amino-Acid Metabolism And Fatty-Acid Metabolism Overview

    This code group represents inherited and acquired disorders that affect branched-chain amino-acid metabolism and fatty-acid oxidation, impacting energy production and leading to metabolic crises or chronic organ dysfunction. These conditions primarily involve metabolic and hepatic systems but can affect the nervous and muscular systems as well. Accurate coding is important for correct claim processing and appropriate documentation of clinical complexity.

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    When to Use

    Use a code from this group when a patient has a documented diagnosis of a branched-chain amino-acid metabolism disorder or a fatty-acid oxidation defect confirmed by biochemical testing or genetic testing. Select the code that best reflects the specific enzyme defect, clinical presentation, and any documented complications or organ involvement.

    ICD-10-CM Codes

    38 rows

    ICD-10-CM CodeDescription
    E71.0Maple-syrup-urine disease
    E71.110Isovaleric acidemia
    E71.1113-methylglutaconic aciduria
    E71.118Other branched-chain organic acidurias
    E71.120Methylmalonic acidemia
    E71.121Propionic acidemia
    E71.128Other disorders of propionate metabolism
    E71.19Other disorders of branched-chain amino-acid metabolism
    E71.2Disorder of branched-chain amino-acid metabolism, unspecified
    E71.30Disorder of fatty-acid metabolism, unspecified
    E71.310Long chain/very long chain acyl CoA dehydrogenase deficiency
    E71.311Medium chain acyl CoA dehydrogenase deficiency
    E71.312Short chain acyl CoA dehydrogenase deficiency
    E71.313Glutaric aciduria type II
    E71.314Muscle carnitine palmitoyltransferase deficiency
    E71.318Other disorders of fatty-acid oxidation
    E71.32Disorders of ketone metabolism
    E71.39Other disorders of fatty-acid metabolism
    E71.40Disorder of carnitine metabolism, unspecified
    E71.41Primary carnitine deficiency

    Showing 1–20 of 38

    DRG Codes

    1 rows

    DRG CodeDescription
    642Inborn and Other Disorders of Metabolism

    Related Codes

    E70-DISORDERS-OF-AROMATIC-AMINO-ACID-METABOLISM

    ICD-10-CM Code For Disorders Of Aromatic Amino-Acid Metabolism: Group Overview

    E72-OTHER-DISORDERS-OF-AMINO-ACID-METABOLISM

    ICD-10-CM Code For Other Disorders Of Amino-Acid Metabolism: Group Overview

    E73-LACTOSE-INTOLERANCE

    ICD-10-CM Code For Lactose Intolerance: Group Overview

    E74-OTHER-DISORDERS-OF-CARBOHYDRATE-METABOLISM

    ICD-10-CM Code For Other Disorders Of Carbohydrate Metabolism: Group Overview

    E75-DISORDERS-OF-SPHINGOLIPID-METABOLISM

    ICD-10-CM Code For Disorders Of Sphingolipid Metabolism And Other Lipid Storage Disorders: Group Overview

    E76-DISORDERS-OF-GLYCOSAMINOGLYCAN-METABOLISM

    ICD-10-CM Code For Disorders Of Glycosaminoglycan Metabolism: Group Overview

    E77-DISORDERS-OF-GLYCOPROTEIN-METABOLISM

    ICD-10-CM Code For Disorders Of Glycoprotein Metabolism: Group Overview

    E78-DISORDERS-OF-LIPOPROTEIN-METABOLISM

    ICD-10-CM Code For Disorders Of Lipoprotein Metabolism And Other Lipidemias: Group Overview

    E79-DISORDERS-OF-PURINE-AND-PYRIMIDINE-METABOLISM

    ICD-10-CM Code For Disorders Of Purine And Pyrimidine Metabolism: Group Overview

    E80-DISORDERS-OF-PORPHYRIN-AND-BILIRUBIN-METABOLISM

    ICD-10-CM Code For Disorders Of Porphyrin And Bilirubin Metabolism: Group Overview

    Showing 1–10 of 16

    Frequently Asked Questions

    References

    1. 1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
    2. 2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
    3. 3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.
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