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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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2018 ICD-10-CM Diagnosis Code E76.22
Sanfilippo mucopolysaccharidoses
2016 2017 2018 Billable/Specific Code
- E76.22 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 E76.22 became effective on October 1, 2017.
- This is the American ICD-10-CM version of E76.22 - other international versions of ICD-10 E76.22 may differ.
Applicable To- Mucopolysaccharidosis, type III (A) (B) (C) (D)
- Sanfilippo A syndrome
- Sanfilippo B syndrome
- Sanfilippo C syndrome
- Sanfilippo D syndrome
The following code(s) above
E76.22 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
E76.22:
- A rare autosomal recessive lysosomal storage disease caused by deficiency of the enzyme acetyl-coa:alpha-glucosaminide acetyltransferase. It is characterized by behavioral changes, sleep disturbances, and mental developmental delays.
- A rare autosomal recessive lysosomal storage disease caused by deficiency of the enzyme heparan sulfate sulfatase. It is characterized by behavioral changes, sleep disturbances, mental developmental delays and seizures.
- A rare autosomal recessive lysosomal storage disease caused by deficiency of the enzyme n-acetyl-alpha-d-glucosaminidase. It is characterized by behavioral changes, sleep disturbances, and mental developmental delays.
- A rare autosomal recessive lysosomal storage disease caused by deficiency of the enzyme n-acetylglucosamine-6-sulfatase. It is characterized by behavioral changes, sleep disturbances and mental developmental delays.
ICD-10-CM E76.22 is grouped within Diagnostic Related Group(s) (MS-DRG v35.0):
- 642 Inborn and other disorders of metabolism
Convert E76.22 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
- Mucopolysaccharidosis E76.3
ICD-10-CM Diagnosis Code E76.3
Mucopolysaccharidosis, unspecified
2016 2017 2018 Billable/Specific Code
- type
- Sanfilippo syndrome E76.22
- Sanfilippo E76.22 (Type B) (Type C) (Type D)
ICD-10-CM Codes Adjacent To E76.22
E76.0 Mucopolysaccharidosis, type I
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
Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.
