2021 ICD-10-CM Diagnosis Code E76.219

Morquio mucopolysaccharidoses, unspecified

    2016 2017 2018 2019 2020 2021 Billable/Specific Code
  • E76.219 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2021 edition of ICD-10-CM E76.219 became effective on October 1, 2020.
  • This is the American ICD-10-CM version of E76.219 - other international versions of ICD-10 E76.219 may differ.
Applicable To
  • Morquio syndrome
  • Mucopolysaccharidosis, type IV
The following code(s) above E76.219 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 E76.219:
  • E00-E89
    2021 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
    2021 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndromes (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
Approximate Synonyms
  • Morquio syndrome
  • Mucopolysaccharidosis mps-iv-a
  • Mucopolysaccharidosis mps-iv-b
Clinical Information
  • A rare autosomal recessive lysosomal storage disease characterized by abnormal skeletal developments, dwarfism, heart disorders, and central nervous system deficits.
  • An inborn error of metabolism characterized by faulty degradation of keratan sulfate with lysosomal accumulation and mucopolysaccharidosis, keratansulfaturia. Clinical features include short trunk dwarfism, dysostosis multiplex, progressive spinal deformity, short neck, pectus carinatum, genu valgum, pes planus, and odontoid hypoplasia with varying degrees of severity. Mental development is usually normal but progressive intellectual deterioration was reported in type b. Two types are recognized according to the enzymes involved: type a: synonyms: morquio syndrome a galactosamine-4-sulfatase (galns) deficiency mucopolysaccharidosis (mps) iv a this type is caused by galactosamine-6-sulfate sulfatase (ec 3.16.4) and is more severe than type b. It is marked by shortness and hyperextension of the neck causing the head to appear as if it were resting directly on the shoulders, short trunk, long extremities with excessive joint mobility, kyphosis or kyphoscoliosis, pectus carinatum, the sternum extending from clavicular junction and angling downward in midsection, spinal cord compression associated with atlantoaxial dislocation and thoracolumbar gibbus, protruding abdomen, and clouding of the cornea type b: synonyms: morquio syndrome b morquio-like syndrome beta-galactosidase deficiency mucopolysaccharidosis (mps) iv b this type is caused by beta-galactosidase (ec 3.2.1.23) deficiency and is marked a milder phenotype consisting of dysostosis multiplex, pectus carinatum, odontoid hypoplasia, kyphosis, genua valga, platyspondyly, and corneal clouding.
  • Genetic disorder of mucopolysaccharide metabolism characterized by skeletal abnormalities, joint instability, development of cervical myelopathy, and excessive urinary keratan sulfate.
  • 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.
ICD-10-CM E76.219 is grouped within Diagnostic Related Group(s) (MS-DRG v38.0):
  • 642 Inborn and other disorders of metabolism

Convert E76.219 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

Diagnosis Index entries containing back-references to E76.219:
  • Chondro-osteodysplasia E76.219 (Morquio-Brailsford type)
  • Kyphosis, kyphotic (acquired) M40.209
    ICD-10-CM Diagnosis Code M40.209

    Unspecified kyphosis, site unspecified

      2016 2017 2018 2019 2020 2021 Billable/Specific Code
    • Morquio-Brailsford type E76.219 (spinal) - see also subcategory M49.8
  • Mucopolysaccharidosis E76.3
    ICD-10-CM Diagnosis Code E76.3

    Mucopolysaccharidosis, unspecified

      2016 2017 2018 2019 2020 2021 Billable/Specific Code

ICD-10-CM Codes Adjacent To E76.219
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
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

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