Wilson's Disease
Wilson’s disease is a genetic disorder that results in excessive accumulation of copper in many parts of the body, particularly the liver. This condition is readily treatable, but if Wilson’s disease is left untreated, it can be fatal.
What are the symptoms of Wilson’s disease?
Wilson’s disease is initially silent and may first be noticed as fatigue, absent menstrual periods in premenopausal women, or repeated and unexplained spontaneous abortions. In more advanced stages, there may be headaches, tremors, uncoordinated limb movements, unsteady gait, drooling, difficulty swallowing, and joint pain. There also may also be strange thought patterns with unusual behaviors.
How is it treated?
Conventional therapy consists of continual and lifelong treatment with medications, such as penicillamine (given with vitamin B6, since it depletes this vitamin), trientine, or dimercaprol, that remove excess copper. Zinc supplements are sometimes used, but not in combination with the prescription medications, because zinc and the medications can interfere with each other.
Dietary changes that may be helpful:
Most foods contain at least some copper, so it is not possible to avoid the metal completely. Foods high in copper, such as organ meats and oysters, should be eliminated from the diet. Some foods are relatively high in copper but are quite nutritious (e.g., nuts and legumes) These foods should be eaten in moderation by people with Wilson’s disease. Grains contain significant amounts of copper but are important components of a healthful diet, and dietary restriction may be neither wise nor necessary, particularly if zinc is supplemented. |
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