2026 ICD-10-CM Diagnosis Code E76.3Mucopolysaccharidosis, unspecified

AHRQ’s CCSR groups this code into broader clinical categories.

• Hunter Syndrome|Mucopolysaccharidosis Type II

  an x-linked, inherited lysosomal storage disease caused by the deficiency of the enzyme iduronate sulfatase that is responsible for the degradation of mucopolysaccharides. it nearly always affects males and is characterized by the accumulation of mucopolysaccharides in various organs, resulting in mental dysfunction, enlarged abdomen, hearing loss, obstructive airway disease, heart disease, and hepatosplenomegaly.

• Hurler Syndrome|MPS I H|Mucopolysaccharidosis Type IH

  an autosomal recessive inherited disorder of mucopolysaccharide metabolism. it is the most severe form of mucopolysaccharidosis type i. it is characterized by deficiency of the enzyme alpha-l-iduronidase resulting in the accumulation of mucopolysaccharides in the tissues.

• IDUA wt Allele|Alpha-L-Iduronidase wt Allele|IDA|Iduronidase Alpha-L- Gene|Iduronidase, Alpha-L Gene|Iduronidase, Alpha-L- Gene|MPS1|MPSI|Mucopolysaccharidosis Type I Gene

  human idua wild-type allele is located in the vicinity of 4p16.3 and is approximately 18 kb in length. this allele, which encodes alpha-l-iduronidase protein, is involved in the metabolism of glycosaminoglycans. mutation of the gene is associated with mucopolysaccharidosis type 1.

• Maroteaux-Lamy Syndrome|Mucopolysaccharidosis Type VI|Mucopolysaccharidosis VI

  a rare autosomal recessive lysosomal storage disease caused by deficiency of the enzyme n-acetylgalactosamine-4-sulfatase. it is characterized by organomegaly, short stature, joint stiffness, otitis media, and respiratory illnesses.

• Morquio Syndrome|Mucopolysaccharidosis IV|Mucopolysaccharidosis Type IV

  a rare autosomal recessive lysosomal storage disease characterized by abnormal skeletal developments, dwarfism, heart disorders, and central nervous system deficits.

• 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, intellectual disabilities, abnormal skeletal development, heart disorders, hearing loss, and central nervous system deficiencies.

• Mucopolysaccharidosis Type I|MPS I|Mucopolysaccharidosis I

  the most common type of mucopolysaccharidosis. it is inherited in an autosomal recessive pattern. it comprises a group of lysosomal storage diseases which includes the most severe form (hurler syndrome) and the mildest form (scheie syndrome).

• Mucopolysaccharidosis Type IIIA|MPS III A|Sanfilippo A

  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.

• Mucopolysaccharidosis Type IIIB|MPS III B|Sanfilippo B

  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.

• Mucopolysaccharidosis Type IIIC|MPS III C|Sanfilippo C

  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.

• Mucopolysaccharidosis Type IIID|MPS III D|Sanfilippo D

  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.

• Mucopolysaccharidosis Type IVA|MPS IV A

  a rare autosomal recessive lysosomal storage disease caused by deficiency of the enzyme galactosamine-6-sulfatase. it is characterized by skeletal and central nervous system deficits.

• Mucopolysaccharidosis Type IVB|MPS IV B

  a rare autosomal recessive lysosomal storage disease caused by deficiency of the enzyme beta galactosidase. it is characterized by skeletal dysplasia and short stature.

• Mucopolysaccharidosis Type IX|Hyaluronidase Deficiency|MPS9

  an autosomal recessive lysosomal storage disease caused by mutation(s) in the hyal1 gene, encoding hyaluronidase-1. it is characterized by short stature and hyaluronidase deficiency.

• Sanfilippo Syndrome|Mucopolysaccharidosis Type III

  a rare autosomal recessive lysosomal storage disease affecting the metabolism of mucopolysaccharides. signs and symptoms include behavioral changes, sleep disorders, mental developmental delays, and seizures.

• Sly Syndrome|Beta-Glucuronidase Deficiency|Mucopolysaccharidosis Type VII

  a rare autosomal recessive lysosomal storage disease caused by deficiency of the enzyme beta-glucuronidase. it is characterized by hepatosplenomegaly, skeletal deformities, enlarged head, and mental retardation.

• SGSH wt Allele|HSS|Heparan Sulfate Sulfatase Gene|MPS3A|Mucopolysaccharidosis Type IIIA Gene|N-Sulfoglucosamine Sulfohydrolase wt Allele|SFMD|Sulfamidase Gene

  human sgsh wild-type allele is located in the vicinity of 17q25.3 and is approximately 20 kb in length. this allele, which encodes n-sulphoglucosamine sulphohydrolase protein, is involved in lysosomal heparan sulfate degradation. mutations in the gene are associated with mucopolysaccharidosis type iiia.

Carbohydrate Metabolism Disorders

Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system (enzymes) break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues.

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