Disorders of glycosaminoglycan metabolism (E76) ICD-10-CM
The E76 code range covers disorders of glycosaminoglycan metabolism with 16 ICD-10-CM diagnosis codes. 12 of them are billable and valid for claim submission in fiscal year 2027, and the category headers group them but cannot themselves be billed.
Codes in the E76 Range 16 codes · 12 billable
- E76 Disorders of glycosaminoglycan metabolismNon-billable
- E76.0 Mucopolysaccharidosis, type INon-billable
- E76.01 Hurler's syndrome
- E76.02 Hurler-Scheie syndrome
- E76.03 Scheie's syndrome
- E76.1 Mucopolysaccharidosis, type II
- E76.2 Other mucopolysaccharidosesNon-billable
- E76.21 Morquio mucopolysaccharidosesNon-billable
- E76.210 Morquio A mucopolysaccharidoses
- E76.211 Morquio B mucopolysaccharidoses
- E76.219 Morquio mucopolysaccharidoses, unspecified
- E76.22 Sanfilippo mucopolysaccharidoses
- E76.29 Other mucopolysaccharidoses
- E76.3 Mucopolysaccharidosis, unspecified
- E76.8 Other disorders of glucosaminoglycan metabolism
- E76.9 Glucosaminoglycan metabolism disorder, unspecified
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the E76 range.
Hurler-Scheie Syndrome
An autosomal recessive disorder representing the intermediate form of mucopolysaccharidosis type I. It is characterized by deficiency of the enzyme alpha-L-iduronidase. Signs and symptoms include short stature, cloudy cornea, umbilical hernia, joint stiffening, hepatosplenomegaly, and mental retardation.
Mucopolysaccharidoses
Group of lysosomal storage diseases each caused by an inherited deficiency of an enzyme involved in the degradation of glycosaminoglycans (mucopolysaccharides). The diseases are progressive and often display a wide spectrum of clinical severity within one enzyme deficiency.
Mucopolysaccharidosis I
A group of autosomal recessive lysosomal storage disorders caused by mutations in the gene encoding the enzyme, alpha-L-iduronidase (IDUA), required for the degradation of heparan and dermatan sulfates. This leads to abnormal accumulation of these glycosaminoglycans in various tissues causing a wide range of clinical presentations including cognitive and musculoskeletal disorders.
Mucopolysaccharidosis II
Systemic lysosomal storage disease marked by progressive physical deterioration and caused by a deficiency of L-sulfoiduronate sulfatase. This disease differs from MUCOPOLYSACCHARIDOSIS I by slower progression, lack of corneal clouding, and X-linked rather than autosomal recessive inheritance. The mild form produces near-normal intelligence and life span. The severe form usually causes death by age 15.
Mucopolysaccharidosis III
Mucopolysaccharidosis characterized by HEPARAN SULFATE in the urine, progressive mental retardation, mild dwarfism, and other skeletal disorders. There are four clinically indistinguishable but biochemically distinct forms, each due to a deficiency of a different enzyme.
Mucopolysaccharidosis IV
Genetic disorder of mucopolysaccharide metabolism characterized by skeletal abnormalities, joint instability, development of cervical myelopathy, and excessive urinary keratan sulfate. There are two biochemically distinct forms, each due to a deficiency of a different enzyme.
Mucopolysaccharidosis VI
Mucopolysaccharidosis with excessive CHONDROITIN SULFATE B in urine, characterized by dwarfism and deafness. It is caused by a deficiency of N-ACETYLGALACTOSAMINE-4-SULFATASE (arylsulfatase B).
Scheie Syndrome
An autosomal recessive disorder representing the milder form of mucopolysaccharidosis type I. It is characterized by deficiency of the enzyme alpha-L-iduronidase. Signs and symptoms include broad mouth with full lips, cloudy cornea which may lead to blindness, stiff joints, and hirsutism.
About the E76 Code Range
These metabolic disorders include named forms of mucopolysaccharidosis and other disorders of glycosaminoglycan metabolism.
E76.0 groups type I mucopolysaccharidosis into Hurler's, Hurler-Scheie and Scheie's syndromes. E76.1 identifies type II. E76.2 groups other mucopolysaccharidoses, including Morquio forms separated into A, B and unspecified, and Sanfilippo mucopolysaccharidoses.
E76.3 identifies unspecified mucopolysaccharidosis. Separate codes identify other and unspecified disorders of glucosaminoglycan metabolism.
Questions About This Page
How many billable codes are in the E76 range?
Of the 16 codes in this range, 12 are billable and valid for claim submission from October 1, 2026 through September 30, 2027. Category header codes group them but cannot be reported on claims.
What does the E76 range classify?
The range classifies disorders of glycosaminoglycan metabolism. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2027 ICD-10-CM Tabular List and order file, effective October 1, 2026 through September 30, 2027.
