2023 ICD-10-CM Diagnosis Code E76.29

Other mucopolysaccharidoses

    2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
  • E76.29 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2023 edition of ICD-10-CM E76.29 became effective on October 1, 2022.
  • This is the American ICD-10-CM version of E76.29 - other international versions of ICD-10 E76.29 may differ.
Applicable To
  • beta-Glucuronidase deficiency
  • Maroteaux-Lamy (mild) (severe) syndrome
  • Mucopolysaccharidosis, types VI, VII
The following code(s) above E76.29 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 E76.29:
  • E00-E89
    2023 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    • 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
    2023 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Type 2 Excludes
    • Ehlers-Danlos syndromes (Q79.6-)
    Metabolic disorders
Approximate Synonyms
  • Maroteaux lamy syndrome
Clinical Information
  • A rare autosomal recessive lysosomal storage disease caused by deficiency of the enzyme beta-glucuronidase. It is characterized by hepatosplenomegaly, skeletal deformities, enlarged head, and mental retardation.
  • An inborn error of metabolism characterized by beta-glucuronidase (ec deficiency with abnormal storage of mucopolysaccharides in various tissues. The phenotype consists mainly of short stature, hepatosplenomegaly, dysostosis multiplex, and mild mental retardation. Type i is the most severe with hydrops fetalis, coarse facies with hypertelorism and depressed nasal bridge, cloudy corneae, and onset of symptoms at birth. Type ii has a less severe course with moderate huurler facies and hypertelorism and onset at 2 to 3 years. Type iii has the mildest symptoms with onset during adolescence.
  • Mucopolysaccharidosis characterized by excessive dermatan and heparan sulfates in the urine and hurler-like features. It is caused by a deficiency of beta-glucuronidase.
ICD-10-CM E76.29 is grouped within Diagnostic Related Group(s) (MS-DRG v40.0):
  • 642 Inborn and other disorders of metabolism

Convert E76.29 to ICD-9-CM

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
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): No change
  • 2023 (effective 10/1/2022): No change

Diagnosis Index entries containing back-references to E76.29:
  • Chondro-osteodystrophy E76.29
  • Deficiency, deficient
    • beta-glucuronidase E76.29
  • Eccentro-osteochondrodysplasia E76.29
  • Maroteaux-Lamy syndrome E76.29 (mild) (severe)
  • Mucopolysaccharidosis E76.3
    ICD-10-CM Diagnosis Code E76.3

    Mucopolysaccharidosis, unspecified

      2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
    • type
      • VI E76.29
      • VII E76.29
    • beta-gluduronidase deficiency E76.29
    • Maroteaux-Lamy syndrome E76.29
    • specified NEC E76.29

ICD-10-CM Codes Adjacent To E76.29
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
E76.29 Other mucopolysaccharidoses
E76.3 Mucopolysaccharidosis, unspecified
E76.8 Other disorders of glucosaminoglycan metabolism
E76.9 Glucosaminoglycan metabolism disorder, unspecified
E77 Disorders of glycoprotein metabolism
E77.0 Defects in post-translational modification of lysosomal enzymes
E77.1 Defects in glycoprotein degradation
E77.8 Other disorders of glycoprotein metabolism
E77.9 Disorder of glycoprotein metabolism, unspecified
E78 Disorders of lipoprotein metabolism and other lipidemias
E78.0 Pure hypercholesterolemia

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