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What is Mucopolysaccharidosis type I Hurler-Scheie syndrome?

What is Mucopolysaccharidosis type I Hurler-Scheie syndrome?

  • Mucopolysaccharidosis type I Hurler-Scheie syndrome: A rare inherited biochemical disorder characterized by the accumulation of mucopolysaccharides (glycosaminoglycans) in various body tissues due to insufficient amounts of the enzyme (?-L-diuronidase) needed to break it down.

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

Mucopolysaccharidosis type I Hurler-Scheie syndrome: Introduction

Types of Mucopolysaccharidosis type I Hurler-Scheie syndrome:

Types of Mucopolysaccharidosis type I Hurler-Scheie syndrome:

Broader types of Mucopolysaccharidosis type I Hurler-Scheie syndrome:

How many people get Mucopolysaccharidosis type I Hurler-Scheie syndrome?

Prevalance of Mucopolysaccharidosis type I Hurler-Scheie syndrome: about 200 people ("Orphan Products: Hope for People With Rare Diseases", By Carol Rados, FDA Consumer magazine, November-December 2003 Issue)
Prevalance Rate of Mucopolysaccharidosis type I Hurler-Scheie syndrome: approx 1 in 1,360,000 or 0.00% or 200 people in USA [about data]

Who gets Mucopolysaccharidosis type I Hurler-Scheie syndrome?

Patient Profile for Mucopolysaccharidosis type I Hurler-Scheie syndrome: Infants.

How serious is Mucopolysaccharidosis type I Hurler-Scheie syndrome?

Prognosis of Mucopolysaccharidosis type I Hurler-Scheie syndrome: Poor. Usually death by age 10.
Complications of Mucopolysaccharidosis type I Hurler-Scheie syndrome: see complications of Mucopolysaccharidosis type I Hurler-Scheie syndrome

What causes Mucopolysaccharidosis type I Hurler-Scheie syndrome?

Causes of Mucopolysaccharidosis type I Hurler-Scheie syndrome: see causes of Mucopolysaccharidosis type I Hurler-Scheie syndrome

What are the symptoms of Mucopolysaccharidosis type I Hurler-Scheie syndrome?

Symptoms of Mucopolysaccharidosis type I Hurler-Scheie syndrome: see symptoms of Mucopolysaccharidosis type I Hurler-Scheie syndrome

Complications of Mucopolysaccharidosis type I Hurler-Scheie syndrome: see complications of Mucopolysaccharidosis type I Hurler-Scheie syndrome

Can anyone else get Mucopolysaccharidosis type I Hurler-Scheie syndrome?

More information: see contagiousness of Mucopolysaccharidosis type I Hurler-Scheie syndrome
Inheritance: see inheritance of Mucopolysaccharidosis type I Hurler-Scheie syndrome

How is it treated?

Doctors and Medical Specialists for Mucopolysaccharidosis type I Hurler-Scheie syndrome: Medical Geneticist, Medical Geneticist ; see also doctors and medical specialists for Mucopolysaccharidosis type I Hurler-Scheie syndrome.
Treatments for Mucopolysaccharidosis type I Hurler-Scheie syndrome: see treatments for Mucopolysaccharidosis type I Hurler-Scheie syndrome
Research for Mucopolysaccharidosis type I Hurler-Scheie syndrome: see research for Mucopolysaccharidosis type I Hurler-Scheie syndrome

Name and Aliases of Mucopolysaccharidosis type I Hurler-Scheie syndrome

Main name of condition: Mucopolysaccharidosis type I Hurler-Scheie syndrome

Other names or spellings for Mucopolysaccharidosis type I Hurler-Scheie syndrome:

MPS I, Mucopolysaccharidosis 1, Mucopolysaccharidosis I, Mucopolysaccharidosis type 1, Mucopolysaccharidosis type I, Alpha-L-iduronidase deficiency, intermediate, MPS 1H/S, Alpha-L-iduronidase deficiency

MPS 1H/S
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)

Mucopolysaccharidosis type I Hurler-Scheie syndrome: Related Conditions

Research the causes of these diseases that are similar to, or related to, Mucopolysaccharidosis type I Hurler-Scheie syndrome:

 

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