Version 2024

2024 ICD-10-CM Diagnosis Code E76.21

Morquio mucopolysaccharidoses

ICD-10-CM Code:
E76.21
ICD-10 Code for:
Morquio mucopolysaccharidoses
Is Billable?
Not Valid for Submission
Code Navigator:

Code Classification

  • Endocrine, nutritional and metabolic diseases
    (E00–E89)
    • Metabolic disorders
      (E70-E88)
      • Disorders of glycosaminoglycan metabolism
        (E76)

E76.21 is a non-specific and non-billable diagnosis code code, consider using a code with a higher level of specificity for a diagnosis of morquio mucopolysaccharidoses. The code is not specific and is NOT valid for the year 2024 for the submission of HIPAA-covered transactions. Category or Header define the heading of a category of codes that may be further subdivided by the use of 4th, 5th, 6th or 7th characters.

Specific Coding Applicable to Morquio mucopolysaccharidoses

Non-specific codes like E76.21 require more digits to indicate the appropriate level of specificity. Consider using any of the following ICD-10-CM codes with a higher level of specificity when coding for morquio mucopolysaccharidoses:

  • Use E76.210 for Morquio A mucopolysaccharidoses - BILLABLE CODE

  • Use E76.211 for Morquio B mucopolysaccharidoses - BILLABLE CODE

  • Use E76.219 for Morquio mucopolysaccharidoses, unspecified - BILLABLE CODE

Clinical Information

  • 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.

Patient Education


Mucopolysaccharidosis type IV

Mucopolysaccharidosis type IV (MPS IV), also known as Morquio syndrome, is a progressive condition that mainly affects the skeleton. The rate at which symptoms worsen varies among affected individuals.

The first signs and symptoms of MPS IV usually become apparent during early childhood. Affected individuals develop various skeletal abnormalities, including short stature, knock knees, and abnormalities of the ribs, chest, spine, hips, and wrists. People with MPS IV often have joints that are loose and very flexible (hypermobile), but they may also have restricted movement in certain joints. A characteristic feature of this condition is underdevelopment (hypoplasia) of a peg-like bone in the neck called the odontoid process. The odontoid process helps stabilize the spinal bones in the neck (cervical vertebrae). Odontoid hypoplasia can lead to misalignment of the cervical vertebrae, which may compress and damage the spinal cord, resulting in paralysis or death.

In people with MPS IV, the clear covering of the eye (cornea) typically becomes cloudy, which can cause vision loss. Some affected individuals have recurrent ear infections and hearing loss. The airway may become narrow in some people with MPS IV, leading to frequent upper respiratory infections and short pauses in breathing during sleep (sleep apnea). Other common features of this condition include mildly "coarse" facial features, thin tooth enamel, multiple cavities, heart valve abnormalities, a mildly enlarged liver (hepatomegaly), and a soft out-pouching around the belly-button (umbilical hernia) or lower abdomen (inguinal hernia). Unlike some other types of mucopolysaccharidosis, MPS IV does not affect intelligence.

The life expectancy of individuals with MPS IV depends on the severity of symptoms. Severely affected individuals may survive only until late childhood or adolescence. Those with milder forms of the disorder usually live into adulthood, although their life expectancy may be reduced. Spinal cord compression and airway obstruction are major causes of death in people with MPS IV.


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Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.