Mucopolysaccharidosis VI (medical condition): A lysosomal storage disorder that is caused by a deficiency of the ability to metabolise glycosaminoglycans.
See also:
Mucopolysaccharidosis VI:
»Introduction: Mucopolysaccharidosis VI
»Symptoms of Mucopolysaccharidosis VI
»Treatments for Mucopolysaccharidosis VI
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: 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).
Source: MeSH 2007
These medical condition or symptom topics may be relevant to medical information for Mucopolysaccharidosis VI:
Mucopolysaccharidosis VI (medical condition): See Mucopolysaccharidosis VI (disease information).
»Introduction: Mucopolysaccharidosis VI
»Symptoms of Mucopolysaccharidosis VI
»Treatments for Mucopolysaccharidosis VI
Mucopolysaccharidosis VI: Mucopolysaccharidosis VI is listed as a type of (or associated with) the following medical conditions in our database:
MPS VI, or Maroteauz-Lamy syndrome, resembles Hurler syndrome. Onset is in infancy, however, intelligence is normal. Individuals may live into the second or third decade. (Source: excerpt from NINDS Mucopolysaccharidoses Information Page: NINDS)
MPS VI, or Maroteauz-Lamy syndrome, resembles Hurler syndrome. Onset is in infancy, however, intelligence is normal. Individuals may live into the second or third decade. (Source: excerpt from NINDS Mucopolysaccharidoses Information Page: NINDS)
These medical disease topics may be related to Mucopolysaccharidosis VI:
Source: Diseases Database
Source - MeSH 2007
Source - MeSH 2007
The following list attempts to classify Mucopolysaccharidosis VI into categories where each line is subset of the next.
Source: Diseases Database
Search to find out more about Mucopolysaccharidosis VI:
|
What do you think about the features of this website? Take our user survey and have your say:
Tools & Services:
Medical Articles:
Search Specialists by State and City
By using this site you agree to our Terms of Use. Information provided on this site is for informational purposes only; it is not intended as a substitute for advice from your own medical team. The information on this site is not to be used for diagnosing or treating any health concerns you may have - please contact your physician or health care professional for all your medical needs. Please see our Terms of Use.
Copyright © 2009 Health Grades Inc. All rights reserved.
