What is Wilson's Disease?
What is Wilson's Disease?
- Wilson's Disease: Wilson disease, or hepatolenticular degeneration, is a neurodegenerative disease of copper metabolism.
- Wilson's Disease: A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS; TREMOR; ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years.
Source - Diseases Database
- Wilson's Disease: a rare inherited disorder of copper metabolism; copper accumulates in the liver and then in the red blood cells and brain.
Source - WordNet 2.1
Wilson's Disease is listed as a "rare disease" by the Office of
Rare Diseases (ORD) of the National Institutes of Health
(NIH). This means that Wilson's Disease, or a subtype of Wilson's Disease,
affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)
Wilson's Disease: Introduction
Types of Wilson's Disease:
Broader types of Wilson's Disease:
How many people get Wilson's Disease?
Prevalance of Wilson's Disease: approximately 1 per 30,000 people suffer from Wilson disease, Genetics Home Reference website
Prevalance Rate of Wilson's Disease: approx 1 in 30,000 or 0.00% or 9,066 people in USA [about data]
Who gets Wilson's Disease?
Patient Profile for Wilson's Disease: Symptoms usually age 6-20 but can be as late as 40 or 50. Most common in puberty.
Profile for Wilson's Disease:
Symptoms usually appear between the
ages of 6 and 20 years, but can begin as late as age 40.
(Source: excerpt from Wilson's Disease: NIDDK)
Gender Ratio for Wilson's Disease: slight male predominance
How serious is Wilson's Disease?
Prognosis of Wilson's Disease: Improvement usually begins 5-6 months after the start of therapy and continues for about 24 months. The deficits present at 24 months are likely to be permanent.
Complications of Wilson's Disease:
see complications of Wilson's Disease
Prognosis of Wilson's Disease:
Wilson's disease requires lifelong treatment. If the disorder is
detected early and treated correctly, a person with Wilson's disease can
enjoy completely normal health.
(Source: excerpt from Wilson's Disease: NIDDK)
...
Without proper treatment, Wilson's disease is generally
fatal, usually by the age of 30. If treatment is begun early enough,
symptomatic recovery is usually complete, and a life of normal length and
quality can be expected.
(Source: excerpt from NINDS Wilson's Disease Information Page: NINDS)
What causes Wilson's Disease?
Causes of Wilson's Disease: see causes of Wilson's Disease
Cause of Wilson's Disease: Genetic disease causes copper buildup that causes brain or liver damage resulting in symptoms.
What are the symptoms of Wilson's Disease?
Symptoms of Wilson's Disease:
see symptoms of Wilson's Disease
Complications of Wilson's Disease:
see complications of Wilson's Disease
Onset of Wilson's Disease: The onset of liver disease is usually at the age of 8-16 years.
Can anyone else get Wilson's Disease?
Inheritance:
see inheritance of Wilson's Disease
Inheritance of genetic conditions: see details in inheritance of genetic diseases.
Wilson's Disease: Testing
Diagnostic testing: see tests for Wilson's Disease.
Misdiagnosis: see misdiagnosis and Wilson's Disease.
How is it treated?
Treatments for Wilson's Disease:
see treatments for Wilson's Disease
Research for Wilson's Disease:
see research for Wilson's Disease
Organs Affected by Wilson's Disease:
Organs and body systems related to Wilson's Disease include:
Name and Aliases of Wilson's Disease
Main name of condition: Wilson's Disease
Class of Condition for Wilson's Disease: genetic autosomal recessive
Other names or spellings for Wilson's Disease:
Wilson Disease, Copper overload, Hepatolenticular degeneration, Kinnier Wilson disease, Westphal Strümpel disease, lenticular progressive degeneration, pseudosclerosis hepatolenticular degeneration, coper storage disease, hepatolenticular degeneration syndrome, WD, WND
Gowers' chorea, Hepatolenticular degeneration, Westphal-Struempell pseudosclerosis
Source - Diseases Database
Hepatolenticular degeneration, Wilson's disease
Source - WordNet 2.1
Hepatolenticular degeneration
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)
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» Next page: Online Medical Textbooks for Wilson's Disease
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