Published on February 4, 2014
Wilson’s Disease “Hepatolenticular Degeneration” A rare inherited disorder...
What is Wilson’s disease? Wilsons disease is a rare inherited disorder, when your body takes in and absorbs too much copper. Normally, copper from the diet is filtered out by the liver and released into bile, which flows out of the body. People who have Wilson disease cannot release copper from the liver.When the copper storage capacity of the liver is exceeded, copper is released into the bloodstream and travels to other organs including the brain, kidneys, and eyes.
What caused it? Wilson’s disease is caused by a mutation in the ATP7B gene. This gene controls your liver and bile production. If both of your parents have the gene you are 25% likely to inherit Wilson’s disease.
Who is this most common in? Wilson’s disease was most common in Eastern Europeans, Sicilians, and Southern Italians, this affects females and males the same, and people between 4 and 40.
What parts of your body are affected? Wilson’s disease can create copper deposits in your liver, brain, kidneys, and eyes destroying the tissue.
What are the Symptoms? Symptoms of Wilson Disease are ● ● ● ● ● ● ● ● ● ● ● Delirium (Confusion) Dementia (Forgetfulness, Easily confused) Ataxia (Difficulty Walking) Abdominal Distention (Enlargement of abdomen) Neuroses (Phobias)(Brain) Vomiting blood (Kidneys failing) Icterus (Jaundice eyes or skin)(Liver failing) Tremors of hands or arms (Brain/Nervous System) Speech impairment (Brain) Weakness Emotional and Behavioral changes (Brain)
What are the Exams/Tests? A test for Wilson’s Disease are a slit-lamp eye examination to see Kayser-Fleischer rings. Another test for Wilson’s disease is physical examination to see nervous system damage, liver or spleen disorders
What are long term effects? After you have Wilson’s disease sometimes there is neurological damage, IQ loss, muscle tremors,loss of memory, coordination, and thinking.
What are treatments? Treatments for Wilson’s Disease are Chelation (Reducing the amount of copper in tissues) Penicillamine, Cuprimine, Depen (Binds copper, increases the release of copper in urine) Zinc Acetate(Galzin) (Blocks copper from being absorbed in intestinal tract)
What type of diet is low copper? A low copper diet includes chocolate, dried fruit, liver, mushrooms, and nuts.
Who discovered it? Wilsons disease is named after Dr. Samuel Kinnier Wilson a neurologist during 1875-1937 Wilson’s disease was properly explained Professor John N The gene ATP7B was discovered by 70’s and 80’s research groups
Bibliography ● http://www.news-medical.net/health/WilsonsDisease.aspx ● http://www.nlm.nih. gov/medlineplus/ency/article/000785.htm
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Wilson's disease, also called Wilson disease or hepatolenticular degeneration, is an autosomal recessive genetic disorder in which copper accumulates in ...
In Wilson's disease, a build-up of copper damages organs including the liver, nervous system, brain, kidneys and eyes.
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Most patients with Wilson disease have high levels of copper in the liver tissue when compared with carriers or with people who do not have Wilson disease.
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Wilson disease is a genetic disorder that is fatal unless detected and treated before serious illness from copper poisoning develops. Wilson disease ...
Wilson Disease National Digestive Diseases Information Clearinghouse. ... caused by Wilson disease, the level of blood copper is often higher than normal.
Wilson's disease is a rare inherited disorder that causes too much copper to accumulate in your liver, brain and other vital organs. Symptoms typically ...
Wilson’s disease is a genetic disorder that is fatal unless ... Copper is an essential trace element and an ... Samuel Alexander Kinnier Wilson, ...
Can Wilson's disease result in permanent ... Most reference labs do not automatically calculate the amount of serum free copper in a Wilson's disease ...