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What is Mucopolysaccharidosis VI?

What is Mucopolysaccharidosis VI?

  • Mucopolysaccharidosis VI: A lysosomal storage disorder that is caused by a deficiency of the ability to metabolise glycosaminoglycans.
  • Mucopolysaccharidosis VI: An inborn error of metabolism characterized by arylsulfatase B (EC 3.1.6.12) deficiency preventing degradation of mucopolysaccharides with their accumulation in soft tissues causing obstructions and compression of the blood vessels, trachea, and peripheral nerves, and disruption of normal bone development, associated with the phenotype similar to that in MPS I but generally normal intelligence and mental retardation reported in a few isolated cases. Three basic types are recognized: Maroteaux-Lamy syndrome type B Synonym: mucopolysaccharidosis (MPS) VI B A mild type marked by usually normal childhood until about 6 years of age when short stature, Legg-Perthes-like changes of the hips, aortic stenosis, spinal deformities, corneal clouding, survival into adulthood. The intermediate type has the phenotype similar to that in mucolipidosis III with coarse Hurler-like facies, stiff joints with decreased mobility, and short stature. The severe type (sometimes designated Maroteaux-Lamy syndrome type A Synonym: mucopolysaccharidosis (MPS) VI A A severe typs usually associated with onset of symptoms in early childhood, a rapidly progressive course, and death in adolescence. Short stature, coarse facies, hyperextended head, corneal clouding, defective hearing, heart abnormalities, and musculoskeletal anomalies are the main characteristics.
    Source - Diseases Database

Mucopolysaccharidosis VI is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Mucopolysaccharidosis VI, or a subtype of Mucopolysaccharidosis VI, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Mucopolysaccharidosis VI: Introduction

Types of Mucopolysaccharidosis VI:

Broader types of Mucopolysaccharidosis VI:

Who gets Mucopolysaccharidosis VI?

Patient Profile for Mucopolysaccharidosis VI: infancy.

How serious is Mucopolysaccharidosis VI?

Prognosis of Mucopolysaccharidosis VI: Poor. Usually life shortening to teens or 20's.
Complications of Mucopolysaccharidosis VI: see complications of Mucopolysaccharidosis VI
Prognosis of Mucopolysaccharidosis VI: Individuals may live into the second or third decade. (Source: excerpt from NINDS Mucopolysaccharidoses Information Page: NINDS)

What causes Mucopolysaccharidosis VI?

Causes of Mucopolysaccharidosis VI: see causes of Mucopolysaccharidosis VI

What are the symptoms of Mucopolysaccharidosis VI?

Symptoms of Mucopolysaccharidosis VI: see symptoms of Mucopolysaccharidosis VI

Complications of Mucopolysaccharidosis VI: see complications of Mucopolysaccharidosis VI

Can anyone else get Mucopolysaccharidosis VI?

More information: see contagiousness of Mucopolysaccharidosis VI
Inheritance: see inheritance of Mucopolysaccharidosis VI

Mucopolysaccharidosis VI: Testing

Misdiagnosis: see misdiagnosis and Mucopolysaccharidosis VI.

How is it treated?

Doctors and Medical Specialists for Mucopolysaccharidosis VI: Medical Geneticist ; see also doctors and medical specialists for Mucopolysaccharidosis VI.
Treatments for Mucopolysaccharidosis VI: see treatments for Mucopolysaccharidosis VI
Research for Mucopolysaccharidosis VI: see research for Mucopolysaccharidosis VI

Name and Aliases of Mucopolysaccharidosis VI

Main name of condition: Mucopolysaccharidosis VI

Other names or spellings for Mucopolysaccharidosis VI:

MPS VI, Maroteauz-Lamy syndrome

Arylsulfatase B deficiency, Maroteaux-Lamy disease Source - Diseases Database

Maroteaux Lamy syndrome, N-acetylgalactosamine-4-sulfatase deficiency, Mucopolysaccharidosis type VI, ARSB deficiency, Arylsulfatase B deficiency, MPS 6
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)

Mucopolysaccharidosis VI: Related Conditions

Research the causes of these diseases that are similar to, or related to, Mucopolysaccharidosis VI:

 

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