Mucopolysaccharidosis III: Individuals with MPS III, or Sanfilippo syndrome, experience progressive dementia and mental deterioration in childhood. ... more about Mucopolysaccharidosis III.
Mucopolysaccharidosis III: Disorder of mucopolysaccharide metabolism in children. More detailed information about the symptoms, causes, and treatments of Mucopolysaccharidosis III is available below.
Read more about symptoms of Mucopolysaccharidosis III
Read more about treatments for Mucopolysaccharidosis III
Read more about Deaths and Mucopolysaccharidosis III.
Read more about complications of Mucopolysaccharidosis III.
Read more about causes of Mucopolysaccharidosis III.
Research the causes of these diseases that are similar to, or related to, Mucopolysaccharidosis III:
Prognosis for Mucopolysaccharidosis III: Poor. Death common in late teens.
More about prognosis of Mucopolysaccharidosis III
Types of Mucopolysaccharidosis III
Related forums and medical stories:
Read about other experiences, ask a question about Mucopolysaccharidosis III, or answer someone else's question, on our message boards:
Individuals with MPS III, or Sanfilippo syndrome, experience progressive dementia and mental deterioration in childhood. Death usually occurs in the late teens. (Source: excerpt from NINDS Mucopolysaccharidoses Information Page: NINDS)
An inborn error of metabolism with a deficiency of enzymes involved in heparan sulfate (HS) degradation. The affected infants appear normal at birth with slowing of development taking place at about one to two years, occasionally not becoming apparent until early school age. Behavioral disorders, mental deterioration, and a loss of motor skills are the principal features. Hirsutism, macrocephaly, and limited joint movements. Four types, each with a different enzyme deficiency, are recognized: A, B, C and D. The phenotype is similar in all four types and consists mainly of some facial coarsening with dull appearance, slightly sunken nasal bridge, and abundant scalp hair. Early development is usually normal, followed between the ages of 2 to 6 years by mainly behavioral disorders with progressive loss of mental and motor skills with spastic diplegia, the patient eventually becoming bedridden. Death usually takes place from 10 to 20 years of age. Type A has the most severe course with the earliest onset and mortality. Type A Synonyms: Sanfilippo syndrome A heparan sulfate sulfatase deficiency mucopolysaccharidosis IIIA (MPS IIIA) Caused by heparan sulfatase (EC 3.10.1.1) deficiency. Type B Synonyms: Sanfilippo syndrome B mucopolysaccharidosis IIIB (MPS IIIB) N-acetyl-alpha-D-glucosaminidase (NAG) deficiency N-acetyl-alpha-D-glucosaminidase (NAG) polymorphism] is caused by Caused by N-acetyl-alpha-D-glucosaminidase (EC 3.2.1.50) deficiency. Type C Synonyms: Sanfilippo syndrome C acetyl-CoA:alpha-glucosamide N-acetyltransferase deficiency mucopolysaccharidosis IIIC (MPS IIIC) Caused by acetyl-CoA:alpha-glucosamide N-acetyltransferase (EC 2.3.13) deficiency. Type D Synonyms: Sanfilippo syndrome D mucopolysaccharidosis IIID (MPS IIID) N-acetylglucosamine-6-sulfate sulfatase deficiency) C Caused by N-acetylglucosamine-6-sulfate sulfatase (3.1.6.14) deficiency. - (Source - Diseases Database)
Mucopolysaccharidosis III is listed as a "rare disease" by the Office of
Rare Diseases (ORD) of the National Institutes of Health
(NIH). This means that Mucopolysaccharidosis III, or a subtype of Mucopolysaccharidosis III,
affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)
Ophanet, a consortium of European partners,
currently defines a condition rare when it affects 1 person per 2,000.
They list Mucopolysaccharidosis III as a "rare disease".
Source - Orphanet
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 © 2011 Health Grades Inc. All rights reserved. Last Update: 1 February, 2012 (5:00)
