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ICD-10-CM Codes
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E00-E89
Endocrine, nutritional and metabolic diseases
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E70-E88
Metabolic disorders
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E76-
Disorders of glycosaminoglycan metabolism
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2020 ICD-10-CM Diagnosis Code E76.3
Mucopolysaccharidosis, unspecified
2016 2017 2018 2019 2020 Billable/Specific Code
- E76.3 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
- The 2020 edition of ICD-10-CM E76.3 became effective on October 1, 2019.
- This is the American ICD-10-CM version of E76.3 - other international versions of ICD-10 E76.3 may differ.
The following code(s) above
E76.3 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
E76.3:
- A group of autosomal recessive or x-linked inherited lysosomal storage disorders affecting the metabolism of mucopolysaccharides, resulting in the accumulation of mucopolysaccharides in the body. Signs and symptoms include organomegaly, mental retardation, abnormal skeletal development, heart disorders, hearing loss, and central nervous system deficiencies.
- Any of a group of lysosomal storage diseases resulting from defects in degradation of glycosaminoglycans, which are excreted in urine and accumulate in tissue.
- 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.
ICD-10-CM E76.3 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
- 642 Inborn and other disorders of metabolism
Convert E76.3 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
Code annotations containing back-references to E76.3:
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Code First: I52
ICD-10-CM Diagnosis Code I52
Other heart disorders in diseases classified elsewhere
2016 2017 2018 2019 2020 Billable/Specific Code Manifestation Code
Code First- underlying disease, such as:
- congenital syphilis (A50.5)
- mucopolysaccharidosis (E76.3)
- schistosomiasis (B65.0-B65.9)
Type 1 Excludes- heart disease (in):
- gonococcal infection (A54.83)
- meningococcal infection (A39.50)
- rheumatoid arthritis (M05.31)
- syphilis (A52.06)
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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
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 2 Excludes- congenital myotonic chondrodystrophy (G71.13)
ICD-10-CM Codes Adjacent To E76.3
E76.1 Mucopolysaccharidosis, type II
E76.2 Other mucopolysaccharidoses
E76.21 Morquio mucopolysaccharidoses
E76.210 Morquio A mucopolysaccharidoses
E76.211 Morquio B mucopolysaccharidoses
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.
