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Wilson's Disease

Written by Trilok Kapur | Medically Reviewed by Dr. Sunil Shroff, MBBS, MS, FRCS (UK), D. Urol (Lond) on Feb 13, 2018
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Other names: Hepatolenticular degeneration, Copper storage disease

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Wilsonís disease is a rare inherited systemic disorder of copper metabolism. Copper is a key ingredient in the development of healthy nerves, bones, collagen, and the skin pigment melanin. Normally any excess is excreted through bile ó a substance produced in your liver. Though, in this disease, copper initially accumulates in the liver from birth onwards, it may not manifest into visible symptoms till around adolescence. They generally occur between 6-45 years of age, but it is possible they may come on in mid-life.

Wilson's Disease

The most visible sign of ĎWilsonís Diseaseí is the rust colored rings around the edges of the cornea.

When the copper has filled the liver completely it causes inflammation, disease and loss of function of the liver totally and also enters other organs like the eyes, brain and kidneys. The range of problems are, a mild elevation of enzymes to total failure of the liver, weight loss, fatigue, abdominal swelling perhaps by fluid accumulation, loss of appetite, and even jaundice.

If you are very young these symptoms are more acute. After teenage there are neurological symptoms which stand out prominently.

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Wilson's disease is a rare disease and affects only 1 in 30,000 of the population. It has equal propensity for men and women in all racial groups.

Wilsonís disease should not be confused with Wilsonís (temperature) syndrome, also called Wilsonís thyroid syndrome or WTS. The syndrome describes as a thyroid-related one that presents with various non-specific thyroid-related symptoms with normal thyroid lab function tests. Some doctors treat it with a thyroid drug called triiodothyronine. However The American Thyroid Association (ATA) does nor believe in its existence as it lacks "well-known and widely-accepted facts" concerning the functions of the thyroid and has raised concerns about the proposed treatments with any drug as this they believe is potentially harmful.

  1. Ala A, Walker AP, Ashkan K, Dooley JS, Schilsky ML (2007). "Wilson's disease". Lancet 369 (9559): 397Ė408. doi:10.1016/S0140-6736(07)60196-2. PMID 17276780.

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Latest Publications and Research on Wilson's Disease


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Comments

SaraH568

Though Wilson Disease is a rare disease, there are those like my daughter and I who are both diagnosed with WD! It is great to bring more awareness and support to this disease. Though my daughter and I both have our WD under control with our zinc regimen - Gluzin - we have many struggles to contend with. Thanks for the WDA!

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