2020 ICD-10-CM Diagnosis Code E76.0

Mucopolysaccharidosis, type I

    2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
  • E76.0 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.
  • The 2020 edition of ICD-10-CM E76.0 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of E76.0 - other international versions of ICD-10 E76.0 may differ.
The following code(s) above E76.0 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E76.0:
  • E00-E89
    2020 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    Note
    • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2020 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndrome (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
Clinical Information
  • Autosomal recessive systemic lysosomal storage disease caused by a deficiency of alpha-l-iduronidase and characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate; there are three recognized phenotypes representing a spectrum of clinical severity from severe to mild: hurler's syndrome, hurler-scheie syndrome and scheie's syndrome (formerly mucopolysaccharidosis v); symptoms may include dwarfism, hepatosplenomegaly, gargoyle-like facies, corneal clouding, cardiac complications, and noisy breathing.
  • Inborn mucopolysaccharide metabolism disorder with alpha-l-iduronidase deficiency in leukocytes and fibroblasts and abnormal mucopolysaccharide incorporation and degradation by fibroblasts. Three types are recognized. All three types have similar laboratory findings, except that the fibroblasts in mps-i-h are heat-stable, whereas in mps-i-s they are heat-labile. Otherwise, the distinction is mainly clpinical. Johnie mcl was the patient in whom the syndrome was observed, hence the synonym johnie mcl syndrome.mucopolysaccharidosis (mps) i-h synonyms: hurler syndrome gargoylism) the most severe of the three types with coarse (gargyloid) facies, accelerated growth from infancy followed by progressive decline in the rate of development, mental retardation, dysostosis multiplex, corneal clouding, and death before the age of 10 years because of pneumonia and heart failure. Some symptoms (hernia, macrocephaly, respiratory infections, and limited hip abduction) become apparent early in infancy but the complete clinical picture develops during the second year of life.mucopolysaccharidosis (mps) i-s synonyms: scheie syndrome late hurler syndrome forme fruste of hurler syndrome ullrich-scheie syndrome spat-hurler syndrome a more moderate form which is marked by corneal opacities, clawhand, aortic valve disease, normal stature, mild or absent intellectual impairment, and nearly normal life span, depending on cardiac complications. The condition is seldom recognized during infancy or early childhood.mucopolysaccharidosis (mps) h/i-s synonyms: hurler-scheie syndrome, phenotype, compound, genetic compound, or syndrome an intermediate form between hurler and scheie syndromes, including short stature, dysostosis multiplex, hepatosplenomegaly, corneal clouding, umbilical or inguinal hernia, generally normal mental development with psychotic symptoms later in life, and death by age 25 years. The symptoms usually become apparent by the age of two years.
  • Systemic lysosomal storage disease caused by a deficiency of alpha-l-iduronidase (iduronidase) and characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. There are three recognized phenotypes representing a spectrum of clinical severity from severe to mild: hurler's syndrome, hurler-scheie syndrome and scheie's syndrome (formerly mucopolysaccharidosis v). Symptoms may include dwarfism, hepatosplenomegaly, gargoyle-like facies, corneal clouding, cardiac complications, and noisy breathing. Hunter syndrome (mucopolysaccharidosis ii) and hurler syndrome were each originally called "gargoylism" because of the coarseness of the facial features of affected individuals.
  • The most common type of mucopolysaccharidosis. It is inherited in an autosomal recessive pattern. It comprises a group of lysosomal storage diseases which includes the most severe form (hurler syndrome) and the mildest form (scheie syndrome).
Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
Code annotations containing back-references to E76.0:
  • Type 1 Excludes: E74
    , Q77
    ICD-10-CM Diagnosis Code E74

    Other disorders of carbohydrate metabolism

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    ICD-10-CM Diagnosis Code Q77

    Osteochondrodysplasia with defects of growth of tubular bones and spine

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    Type 2 Excludes
    • congenital myotonic chondrodystrophy (G71.13)
ICD-10-CM Codes Adjacent To E76.0
E75.248 Other Niemann-Pick disease
E75.249 …… unspecified
E75.25 Metachromatic leukodystrophy
E75.26 Sulfatase deficiency
E75.29 Other sphingolipidosis
E75.3 Sphingolipidosis, unspecified
E75.4 Neuronal ceroid lipofuscinosis
E75.5 Other lipid storage disorders
E75.6 Lipid storage disorder, unspecified
E76 Disorders of glycosaminoglycan metabolism
E76.0 Mucopolysaccharidosis, type I
E76.01 Hurler's syndrome
E76.02 Hurler-Scheie syndrome
E76.03 Scheie's syndrome
E76.1 Mucopolysaccharidosis, type II
E76.2 Other mucopolysaccharidoses
E76.21 Morquio mucopolysaccharidoses
E76.210 Morquio A mucopolysaccharidoses
E76.211 Morquio B mucopolysaccharidoses
E76.219 …… unspecified
E76.22 Sanfilippo mucopolysaccharidoses

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.