E76.22

Applicable To

• Mucopolysaccharidosis, type III A) B) C) D)

• Sanfilippo A syndrome

• Sanfilippo B syndrome

• Sanfilippo C syndrome

• Sanfilippo D syndrome

Approximate Synonyms

• Sanfilippo syndrome

Clinical Information

• 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.

Details

• E76.22 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM E76.22 became effective on October 1, 2022.

• This is the American ICD-10-CM version of E76.22 – other international versions of ICD-10 E76.22 may differ.