2024 ICD-10-CM Diagnosis Code E76.3

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

• Cardiomyopathy due to mucopolysaccharidosis
• Cardiomyopathy due to mucopolysaccharidosis
• Cardiomyopathy due to mucopolysaccharidosis
• Cerebral degeneration in mucopolysaccharidosis
• Dilated cardiomyopathy due to metabolic disorder
• Dilated cardiomyopathy due to mucopolysaccharidosis
• Mucopolysaccharidosis
• Mucopolysaccharidosis, MPS-I
• Restrictive cardiomyopathy due to mucopolysaccharidosis
• Secondary restrictive cardiomyopathy

Clinical Information

• 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.

Convert E76.3 to ICD-9-CM

• ICD-9-CM Code: 277.5 - Mucopolysaccharidosis
  Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Patient Education

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. If you have a metabolic disorder, something goes wrong with this process.

Carbohydrate metabolism disorders are a group of metabolic disorders. Normally your enzymes break carbohydrates down into glucose (a type of sugar). If you have one of these disorders, you may not have enough enzymes to break down the carbohydrates. Or the enzymes may not work properly. This causes a harmful amount of sugar to build up in your body. That can lead to health problems, some of which can be serious. Some of the disorders are fatal.

These disorders are inherited. Newborn babies get screened for many of them, using blood tests. If there is a family history of one of these disorders, parents can get genetic testing to see whether they carry the gene. Other genetic tests can tell whether the fetus has the disorder or carries the gene for the disorder.

Treatments may include special diets, supplements, and medicines. Some babies may also need additional treatments, if there are complications. For some disorders, there is no cure, but treatments may help with symptoms.

[Learn More in MedlinePlus]

Code History

• FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.