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    v1.0.28
    OpenPayerBilling CodesMetabolic disordersICD-10-CM e76-disorders-of-glycosaminoglycan-metabolism
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    ICD-10-CM Code For Disorders Of Glycosaminoglycan Metabolism: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group E76 includes inherited and acquired disorders of glycosaminoglycan metabolism such as mucopolysaccharidoses and related lysosomal storage diseases that affect connective tissue and multiple organ systems. These ICD-10-CM diagnosis codes are used to document disease manifestations, organ system involvement, and clinical severity to capture the full clinical picture for patients with glycosaminoglycan metabolism disorders. Accurate coding within the ICD-10-CM E76 group supports appropriate reimbursement, complete clinical documentation, and proper claim adjudication.

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    ICD-10-CM E76: Disorders Of Glycosaminoglycan Metabolism Overview

    This code group represents inherited and metabolic disorders affecting glycosaminoglycan synthesis and degradation, commonly involving lysosomal storage and connective tissue function. These conditions impact multiple organ systems including skeletal, neurologic, and cardiovascular systems. Accurate coding is important for complete clinical documentation and for appropriate reimbursement determination.

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    When to Use

    Use a code from this group when a confirmed diagnosis or clinically justified documentation indicates a disorder of glycosaminoglycan metabolism or a specific mucopolysaccharidosis subtype. Apply codes to reflect documented disease severity, complications, and organ system involvement to ensure complete clinical and billing records.

    ICD-10-CM Codes

    12 rows

    ICD-10-CM CodeDescription
    E76.01Hurler's syndrome
    E76.02Hurler-Scheie syndrome
    E76.03Scheie's syndrome
    E76.1Mucopolysaccharidosis, type II
    E76.210Morquio A mucopolysaccharidoses
    E76.211Morquio B mucopolysaccharidoses
    E76.219Morquio mucopolysaccharidoses, unspecified
    E76.22Sanfilippo mucopolysaccharidoses
    E76.29Other mucopolysaccharidoses
    E76.3Mucopolysaccharidosis, unspecified
    E76.8Other disorders of glucosaminoglycan metabolism
    E76.9Glucosaminoglycan metabolism disorder, unspecified

    DRG Codes

    1 rows

    DRG CodeDescription
    642Inborn and Other Disorders of Metabolism

    Typical Clinical Scenarios

    • An infant presents to the pediatric genetics clinic with developmental delay, coarse facial features, hepatosplenomegaly, and recurrent upper respiratory infections. Initial metabolic screening and urine glycosaminoglycan analysis are abnormal, and enzyme assay confirms a mucopolysaccharidosis (MPS) subtype, prompting assignment of a specific disorder of glycosaminoglycan metabolism for ongoing management and genetic counseling. Typical codes: E76.01, E76.02
    • A school-age child with progressive joint stiffness, decreased range of motion, and chronic pain is evaluated by pediatric rheumatology; radiographs show dysostosis multiplex and glycosaminoglycan urine testing supports a diagnosis of an MPS that primarily affects the skeletal system. Because this is a chronic, multisystem inherited metabolic disorder requiring long-term surveillance and supportive therapies, a code from this group is used to document the underlying metabolic etiology contributing to musculoskeletal disability. Typical codes: E76.03, E76.22
    • An adult with a known mucopolysaccharidosis presents to the emergency department with acute respiratory distress from airway obstruction related to cervical spine instability and soft tissue glycosaminoglycan deposition. The acute care team documents the metabolic disorder as a comorbidity contributing to the airway emergency to guide perioperative planning and specialist consultation. Typical codes: E76.29, E76.3

    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

    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

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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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