Wilson Disease

Wilson disease is a rare inherited disorder that causes your body to retain copper. Normally, your liver releases copper it doesn't need into bile, a digestive fluid. With Wilson disease, this does not happen. Copper builds up in your liver and injures liver tissue. Over time, the damage causes your liver to release the copper directly into your bloodstream. The blood carries copper all over your body. Too much copper can damage your kidneys, liver, brain and eyes.

Usually the build-up of copper starts after birth. Symptoms usually start between ages 6 and 20, but can begin as late as age 40. The most characteristic sign is a rusty brown ring around the cornea of the eye.

If you have Wilson disease, you will have to take medicine and follow a low-copper diet for the rest of your life. With early detection and proper treatment, a person with Wilson disease can enjoy normal health.

National Institute of Diabetes and Digestive and Kidney Diseases

Wilson's Disease on Wikipedia

'''Wilson's disease''' is an autosomal recessive hereditary disease, with an incidence of about 1 in 30,000. Its main feature is accumulation of copper in biological tissue, which manifests itself with neurology symptoms and liver disease. The estimated heterozygous Asymptomatic_carrier rate is about 1 in 90, meaning that 1 in 90 people are unaffected carriers of this mutation. The disease affects men and women equally and occurs in all races.

Description

The ''Wilson's disease gene'' (WND) has been mapped to chromosome 13 (13q14.3) and is expressed primarily in the liver, kidney, and placenta but has also been found in the heart, brain, and lung, albeit at much lower levels. The gene codes for a P-type ATPase that transports copper into bile and incorporates it into ceruloplasmin. Bile is a liquid produced by the liver that helps with digestion. The mutant form of WND expressed in people with Wilson's disease inhibits the release of copper into bile. As the excretion of copper from the body is thus impaired, the copper builds up in the liver and injures liver tissue. Eventually, the damage causes the liver to release the copper directly into the bloodstream, which carries the copper throughout the body. The copper buildup leads to damage in the kidneys, brain, and eyes. If not treated, Wilson's disease can cause severe brain damage, liver failure, and death.

Symptoms and signs

Symptoms usually appear between the ages of 6 and 20 years, but sometimes not until the age of 30, and in rare instances up to age 50. The most classical sign are the Kayser-Fleischer rings (brown rings around the cornea in the eye) that result from copper deposition in Descemet's membrane of the cornea. Other signs depend on whether the damage occurs in the liver, blood, central nervous system, urinary system, or musculoskeletal system. Many ... [ Read More ]

External Resources

Tempo Blues Booking Agency - Booking U.P. Wilson, Franck Goldwasser, Benoit Blue Boy, Lazy Lester, Tommy Schneiller, Fillmore Slim, The Super Down Home Trio, Boogie Disease, and other Blues artists.

7 Debilitating Diseases of Business Websites - Illnesses make websites inefficient. The cures, by Dr. Ralph F. Wilson:Web Marketing Today, Issue 41, February 1, 1998.

Tau and alpha-synuclein in neurodegenerative diseases - Article by B. I. Giasson, C. A. Wilson, J. Q. Trojanowski, and V. M.-Y. Lee (University of Pennsylvania)

Wilson Disease and Menkes Disease - Progress in normal and disordered copper homeostasis, wilson disease, menkes disease, aceruloplasminemia, basic and clinical research. 8th International Conference, April 16 - 18, 2001.

Wilson's Disease Association - Learn about this disease, about the association, resources for consultation, diagnosis and treatment. Also copper content in various foods.

Wilson's Disease - Vanderbilt Medical Center takes an in depth look at this disease. Diagnoses, laboratory studies and treatment are discussed.

Wilson's Disease - A definition of this disease, clinical manifestations, diagnosis and treatment.

Wilson Disease - In-depth look at this disease, its symptoms, the causes, diagnosis and treatment.

Wilsons Disease - A look at this disease and the causes, symptoms and treatment. Also a brief article on Dr. Samuel Wilson.

Wilson's Disease Association - Information about Wilson's Disease.

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