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Mucopolysaccharidosis type I Hurler-Scheie syndrome

Mucopolysaccharidosis type I Hurler-Scheie syndrome: Introduction

Mucopolysaccharidosis type I Hurler-Scheie syndrome: Mucopolysaccharidosis I(MPS I), which includes Hurler, Scheie, and Hurler/Scheie syndromes. Hurler syndrome-the most severe form of the ... more about 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. More detailed information about the symptoms, causes, and treatments of Mucopolysaccharidosis type I Hurler-Scheie syndrome is available below.

Symptoms of Mucopolysaccharidosis type I Hurler-Scheie syndrome

Treatments for Mucopolysaccharidosis type I Hurler-Scheie syndrome

  • Symptomatic and supportive
  • Enzyme replacement therapy
  • There is no cure but symptomatic measures can improve quality of life. Bone marrow transplants can provide significant benefits for patients with more serious forms of the disease. Early diagnosis and treatment helps slow the progression of the condition. Genetic counseling is recommended
  • more treatments...»

Wrongly Diagnosed with Mucopolysaccharidosis type I Hurler-Scheie syndrome?

Mucopolysaccharidosis type I Hurler-Scheie syndrome: Deaths

Read more about Deaths and Mucopolysaccharidosis type I Hurler-Scheie syndrome.

Types of Mucopolysaccharidosis type I Hurler-Scheie syndrome

Mucopolysaccharidosis type I Hurler-Scheie syndrome: Complications

Review possible medical complications related to Mucopolysaccharidosis type I Hurler-Scheie syndrome:

  • Compression of spinal cord may lead to neurological problems
  • Death usually occurs by third decade
  • Death - can occur in the first decade in severe cases
  • more complications...»

Causes of Mucopolysaccharidosis type I Hurler-Scheie syndrome

Read more about causes of Mucopolysaccharidosis type I Hurler-Scheie syndrome.

Disease Topics Related To Mucopolysaccharidosis type I Hurler-Scheie syndrome

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

Prognosis for Mucopolysaccharidosis type I Hurler-Scheie syndrome

Prognosis for Mucopolysaccharidosis type I Hurler-Scheie syndrome: Poor. Usually death by age 10.

Research about Mucopolysaccharidosis type I Hurler-Scheie syndrome

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Statistics for Mucopolysaccharidosis type I Hurler-Scheie syndrome

Mucopolysaccharidosis type I Hurler-Scheie syndrome: Broader Related Topics

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Article Excerpts about Mucopolysaccharidosis type I Hurler-Scheie syndrome

Mucopolysaccharidosis I(MPS I), which includes Hurler, Scheie, and Hurler/Scheie syndromes. Hurler syndrome-the most severe form of the mucopolysaccharidoses -occurs in infancy with symptoms such as clouding of the cornea and progressive physical and mental disability. Death usually occurs before age 10. Patients with Scheie syndrome experience milder symptoms and have normal intelligence, while those with Hurler/Scheie syndrome suffer from intermediate symptoms. (Source: excerpt from NINDS Mucopolysaccharidoses Information Page: NINDS)

Definitions of Mucopolysaccharidosis type I Hurler-Scheie syndrome:

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)

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More information about Mucopolysaccharidosis type I Hurler-Scheie syndrome

  1. Mucopolysaccharidosis type I Hurler-Scheie syndrome: Introduction
  2. Symptoms
  3. Causes
  4. Treatments
  5. Types
  6. Deaths
  7. Complications
  8. Prognosis
 

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