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    v1.0.28
    OpenPayerBilling CodesMetabolic disordersICD-10-CM e80-disorders-of-porphyrin-and-bilirubin-metabolism
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    ICD-10-CM Code For Disorders Of Porphyrin And Bilirubin Metabolism: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group E80 includes porphyrias and disorders of bilirubin metabolism such as congenital or acquired defects in porphyrin synthesis and bilirubin conjugation that can cause neurovisceral attacks, photosensitivity, or jaundice. These ICD-10-CM diagnosis codes are used to document clinical scenarios including acute neurovisceral crises, chronic cutaneous photosensitivity, hepatic involvement, or neonatal hyperbilirubinemia, capturing disease severity and organ system involvement. Accurate coding within the ICD-10-CM E80 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM E80: Disorders Of Porphyrin And Bilirubin Metabolism Overview

    The ICD-10-CM E80 group covers inherited and acquired disorders affecting porphyrin synthesis and bilirubin metabolism, including porphyrias and bilirubin conjugation defects. These conditions primarily affect hepatic and hematologic systems and can present with neurovisceral, cutaneous, or jaundice-related manifestations. Accurate coding matters for appropriate reimbursement because it documents disease complexity and supports medical necessity for specialized diagnostics and treatments.

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    When to Use

    Use a code from this group when clinical documentation indicates a specific porphyria subtype, bilirubin conjugation disorder, or related metabolic defect affecting porphyrin or bilirubin pathways. Assign codes to reflect acute versus chronic manifestations, organ system involvement, and any documented complications such as neurovisceral attacks or significant hyperbilirubinemia.

    ICD-10-CM Codes

    10 rows

    ICD-10-CM CodeDescription
    E80.0Hereditary erythropoietic porphyria
    E80.1Porphyria cutanea tarda
    E80.20Unspecified porphyria
    E80.21Acute intermittent (hepatic) porphyria
    E80.29Other porphyria
    E80.3Defects of catalase and peroxidase
    E80.4Gilbert syndrome
    E80.5Crigler-Najjar syndrome
    E80.6Other disorders of bilirubin metabolism
    E80.7Disorder of bilirubin metabolism, unspecified

    DRG Codes

    4 rows

    DRG CodeDescription
    441Disorders of Liver Except Malignancy, Cirrhosis or Alcoholic Hepatitis with MCC
    442Disorders of Liver Except Malignancy, Cirrhosis or Alcoholic Hepatitis with CC
    443Disorders of Liver Except Malignancy, Cirrhosis or Alcoholic Hepatitis without CC/MCC
    642Inborn and Other Disorders of Metabolism

    Typical Clinical Scenarios

    • A newborn in the nursery with visible jaundice and markedly elevated unconjugated bilirubin on laboratory testing; the neonate is evaluated for causes of hyperbilirubinemia after feeding issues and prolonged jaundice. A diagnosis from this group is assigned when inherited defects of bilirubin conjugation or transport are suspected as the cause of persistent neonatal or infantile unconjugated hyperbilirubinemia. Typical codes: E80.2.
    • An adult with a lifelong history of photosensitivity, abdominal pain attacks, and dark urine presents to clinic for chronic management and confirmatory biochemical testing; genetic testing and porphyrin studies suggest a specific porphyria disorder. A code from this group is used to document the inherited or acquired porphyria subtype responsible for recurrent neurovisceral and cutaneous manifestations. Typical codes: E80.0, E80.1.
    • A patient admitted to the hospital with acute hemolytic anemia and marked unconjugated hyperbilirubinemia is found to have secondary impairment of bilirubin metabolism due to liver dysfunction and a concurrent porphyrin disorder complicating the clinical course. A code from this group is assigned to capture the comorbid porphyrin or bilirubin metabolism disorder contributing to laboratory abnormalities and influencing management. Typical codes: E80.3, E80.5.

    Related Codes

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

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

    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

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    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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