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    v1.0.28
    OpenPayerBilling CodesMetabolic disordersICD-10-CM e70-disorders-of-aromatic-amino-acid-metabolism
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    ICD-10-CM Code For Disorders Of Aromatic Amino-Acid Metabolism: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group E70 includes disorders of aromatic amino-acid metabolism such as phenylketonuria and related defects in phenylalanine, tyrosine, and tryptophan metabolism that can cause neurologic and systemic manifestations. These ICD-10-CM diagnosis codes are used to document clinical scenarios including congenital metabolic defects, biochemical severity, and complications such as developmental delay or neurologic impairment. Accurate coding within the ICD-10-CM E70 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM E70: Disorders Of Aromatic Amino-Acid Metabolism Overview

    The ICD-10-CM E70 code group covers inherited and acquired disorders affecting the metabolism of aromatic amino acids, primarily phenylalanine, tyrosine, and tryptophan, which can impact neurologic and systemic function. These conditions target metabolic pathways and often present in pediatric populations but can be relevant across the lifespan. Accurate coding matters for reimbursement because it links specific metabolic diagnoses to medical necessity, treatment complexity, and appropriate claim adjudication.

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    When to Use

    Use a code from this group when documenting a confirmed diagnosis of an aromatic amino-acid metabolic disorder identified by biochemical testing or genetic confirmation. Codes are appropriate when clinical notes describe disease severity, associated complications, or ongoing management tied to the metabolic defect.

    ICD-10-CM Codes

    26 rows

    ICD-10-CM CodeDescription
    E70.0Classical phenylketonuria
    E70.1Other hyperphenylalaninemias
    E70.20Disorder of tyrosine metabolism, unspecified
    E70.21Tyrosinemia
    E70.29Other disorders of tyrosine metabolism
    E70.30Albinism, unspecified
    E70.310X-linked ocular albinism
    E70.311Autosomal recessive ocular albinism
    E70.318Other ocular albinism
    E70.319Ocular albinism, unspecified
    E70.320Tyrosinase negative oculocutaneous albinism
    E70.321Tyrosinase positive oculocutaneous albinism
    E70.328Other oculocutaneous albinism
    E70.329Oculocutaneous albinism, unspecified
    E70.330Chediak-Higashi syndrome
    E70.331Hermansky-Pudlak syndrome
    E70.338Other albinism with hematologic abnormality
    E70.339Albinism with hematologic abnormality, unspecified
    E70.39Other specified albinism
    E70.40Disorders of histidine metabolism, unspecified

    Showing 1–20 of 26

    DRG Codes

    1 rows

    DRG CodeDescription
    642Inborn and Other Disorders of Metabolism

    Typical Clinical Scenarios

    • An infant is brought to the emergency department with poor feeding, vomiting, progressive lethargy, and seizures after a few days of life; newborn metabolic screening results return markedly elevated phenylalanine levels and confirm classic phenylketonuria requiring immediate metabolic consult and dietary management. A diagnosis from this group is assigned because the presentation reflects an inborn error of aromatic amino-acid metabolism detected by newborn screening with acute neurologic risk. Typical codes: E70.0, E70.9.
    • A school-aged child with a history of treated phenylketonuria presents for routine follow-up with developmental assessment and dietary monitoring; the visit documents chronic management, lifelong dietary restriction of phenylalanine, and counseling on adjunct treatments for persistent hyperphenylalaninemia. A code from this group is used to capture the established inherited metabolic disorder and ongoing outpatient management. Typical codes: E70.0, E70.21.
    • An adult patient with long-standing liver disease develops secondary disturbances in tryptophan metabolism with pellagra-like dermatitis and neuropsychiatric symptoms; evaluation attributes the findings to secondary aromatic amino-acid metabolism disorder due to hepatic dysfunction, necessitating treatment of the underlying liver disease and nutritional supplementation. A diagnosis code from this group is assigned to indicate the metabolic disorder secondary to another condition. Typical codes: E70.41, E70.49.

    Related Codes

    E71-DISORDERS-OF-BRANCHED-CHAIN-AMINO-ACID-METABOLISM

    ICD-10-CM Code For Disorders Of Branched Chain Amino Acid Metabolism And Fatty 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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