1. ICD-10-CM Codes
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2. E00-E89 Endocrine, nutritional and metabolic diseases
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3. E70-E88 Metabolic disorders
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4. E76- Disorders of glycosaminoglycan metabolism
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2018 ICD-10-CM Diagnosis Code E76.3

Mucopolysaccharidosis, unspecified

2016 2017 2018 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 2018 edition of ICD-10-CM E76.3 became effective on October 1, 2017.
• This is the American ICD-10-CM version of E76.3 - other international versions of ICD-10 E76.3 may differ.

• Mucopolysaccharidosis

• 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.

• 642 Inborn and other disorders of metabolism

Convert E76.3 to ICD-9-CM

• 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

• Code First: I52
  ICD-10-CM Diagnosis Code I52

  Other heart disorders in diseases classified elsewhere

  2016 2017 2018 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)
• Type 1 Excludes: E74
  , Q77
  ICD-10-CM Diagnosis Code E74

  Other disorders of carbohydrate metabolism

  2016 2017 2018 Non-Billable/Non-Specific Code

  Type 1 Excludes

  • diabetes mellitus (E08-E13)
  • hypoglycemia NOS (E16.2)
  • increased secretion of glucagon (E16.3)
  • mucopolysaccharidosis (E76.0-E76.3)
  ICD-10-CM Diagnosis Code Q77

  Osteochondrodysplasia with defects of growth of tubular bones and spine

  2016 2017 2018 Non-Billable/Non-Specific Code

  Type 1 Excludes

  • mucopolysaccharidosis (E76.0-E76.3)

  Type 2 Excludes

  • congenital myotonic chondrodystrophy (G71.13)

• Cardiopathy I51.9
  - see also Disease, heart
  ICD-10-CM Diagnosis Code I51.9

  Heart disease, unspecified

  2016 2017 2018 Billable/Specific Code
  • mucopolysaccharidosis E76.3
• Mucopolysaccharidosis E76.3
  • cardiopathy E76.3

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