ICD-10-CM Code E76.3

Mucopolysaccharidosis, unspecified

Version 2021 Billable Code

Valid for Submission

E76.3 is a billable code used to specify a medical diagnosis of mucopolysaccharidosis, unspecified. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code E76.3 might also be used to specify conditions or terms like cerebral degeneration in mucopolysaccharidosis, dilated cardiomyopathy secondary to metabolic disorder, dilated cardiomyopathy secondary to mucopolysaccharidosis, mucopolysaccharidosis, mucopolysaccharidosis cardiomyopathy, mucopolysaccharidosis, mps-i, etc

Short Description:Mucopolysaccharidosis, unspecified
Long Description:Mucopolysaccharidosis, unspecified

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code E76.3 are found in the index:


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

  • Cerebral degeneration in mucopolysaccharidosis
  • Dilated cardiomyopathy secondary to metabolic disorder
  • Dilated cardiomyopathy secondary to mucopolysaccharidosis
  • Mucopolysaccharidosis
  • Mucopolysaccharidosis cardiomyopathy
  • Mucopolysaccharidosis, MPS-I
  • Nutritional and metabolic cardiomyopathies
  • Restrictive cardiomyopathy secondary to mucopolysaccharidosis
  • Secondary restrictive cardiomyopathy

Clinical Information

  • MUCOPOLYSACCHARIDOSIS I-. systemic lysosomal storage disease caused by a deficiency of alpha l iduronidase iduronidase and characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. there are three recognized phenotypes representing a spectrum of clinical severity from severe to mild: hurler syndrome hurler scheie syndrome and scheie syndrome formerly mucopolysaccharidosis v. symptoms may include dwarfism; hepatosplenomegaly; thick coarse facial features with low nasal bridge; corneal clouding; cardiac complications; and noisy breathing.
  • MUCOPOLYSACCHARIDOSES-. 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.
  • MUCOPOLYSACCHARIDOSIS III-. mucopolysaccharidosis characterized by heparitin sulfate in the urine progressive mental retardation mild dwarfism and other skeletal disorders. there are four clinically indistinguishable but biochemically distinct forms each due to a deficiency of a different enzyme.
  • MUCOPOLYSACCHARIDOSIS IV-. genetic disorder of mucopolysaccharide metabolism characterized by skeletal abnormalities joint instability development of cervical myelopathy and excessive urinary keratan sulfate. there are two biochemically distinct forms each due to a deficiency of a different enzyme.
  • MUCOPOLYSACCHARIDOSIS VI-. mucopolysaccharidosis with excessive chondroitin sulfate b in urine characterized by dwarfism and deafness. it is caused by a deficiency of n acetylgalactosamine 4 sulfatase arylsulfatase b.
  • MUCOPOLYSACCHARIDOSIS II-. systemic lysosomal storage disease marked by progressive physical deterioration and caused by a deficiency of l sulfoiduronate sulfatase. this disease differs from mucopolysaccharidosis i by slower progression lack of corneal clouding and x linked rather than autosomal recessive inheritance. the mild form produces near normal intelligence and life span. the severe form usually causes death by age 15.
  • MUCOPOLYSACCHARIDOSIS VII-. mucopolysaccharidosis characterized by excessive dermatan and heparan sulfates in the urine and hurler like features. it is caused by a deficiency of beta glucuronidase.

Convert E76.3 to ICD-9

  • 277.5 - Mucopolysaccharidosis (Approximate Flag)

Code Classification

  • Endocrine, nutritional and metabolic diseases (E00–E90)
    • Metabolic disorders (E70-E88)
      • Disorders of glycosaminoglycan metabolism (E76)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

Information for Patients

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.

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