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il 2020-01-29 11:30:56.0 alle 2020-01-29 11:30:56.0
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Wilson's deaseas
Wilson's Disease
Mental symptoms of Wilson's disease

Personality change

Irritability

Aggression

Homicidal or suicidal behavior

Depression

Erratic behavior
Paranoia
Delusional disorders
Unusual sexual behavior (including pedophilia)
Inappropriate behavior (especially sexual)
Cognitive impairment
Deteriorating performance at school
Difficulty concentrating
Insomnia
Fatigue
Wilson's Disease affects approximately 1 in 30,000 people worldwide, making it a rare disorder. However, it is estimated that at least half of the people with Wilson's Disease are never diagnosed, and therefore will suffer, and sometimes die, from the disease.

Wilson's is a genetic disorder that causes the body to retain copper. The liver of a person who has the disease cannot release copper into bile as it should. Over time, the copper reaches a toxic level and injures liver tissue. Eventually, this damage will result in high levels of copper in the bloodstream, which leads to damage in the kidneys, brain, and eyes. Untreated, high copper will eventually cause liver failure and brain damage.

Wilson's Disease occurs equally in men and women. Although some cases of Wilson's Disease can occur due to spontaneous genetic mutation, most cases are transmitted from generation-to-generation. The responsible gene is called ATP7B and is located on chromosome 13.

In order to inherit the disease, both parents must carry the gene. Siblings of Wilson's Disease patients have a one-in-four chance of having the disease. Since both of a siblings' parents are carriers, one-fourth of the siblings' children have the disease, one-half are carriers, and one-fourth are disease-free and carry no Wilson's Disease gene.

Children of Wilson’s patients have a one-in-two-hundred chance of having the disease. A child of a Wilson's Disease patient has a 100 percent chance of getting one abnormal gene. The patient's spouse has a one-in-one-hundred chance of carrying the abnormal Wilson's Disease gene, and half the time, he or she will pass it on. For this reason, all siblings and children of Wilson's Disease patients should be tested for Wilson's Disease. Other relatives who have had symptoms or laboratory tests that indicate liver or neurological disease also should also be tested.

Because Wilson's Disease is often mistaken for other maladies such as multiple sclerosis, Parkinson's disease, or psychiatric problems, medical specialists estimate that only about one thousand cases per year are ever diagnosed. In the early stages of the disease, especially when psychological symptoms occur, the diagnosis is often missed. In a study of misdiagnosis, Wilson’s was diagnosed in only one out of one-hundred-twenty-four patients. The delay between symptoms and diagnosis ranged from one to five years.

The most common of behavioral symptoms of Wilson’s Disease include personality changes, especially irritability and a low threshold to anger. Depression sometimes leading to suicidal ideation and suicide attempts is common. Deteriorating academic and work performance is present in almost all patients. Interestingly, many Wilson's Disease patients exhibit increased sexual preoccupation and reduced sexual inhibition. It is also linked with pedophilia.

A barrier to the diagnosis of Wilson's Disease is that most patients have no family history of it. Because both parents must carry the gene to manifest the disorder, people with only one abnormal gene usually have no symptoms, or may have mild, but medically insignificant, abnormalities of copper metabolism, and do not become ill.

People with Wilson's Disease may not have any outward signs, symptoms, or evidence of illness. However, people with mild or non-apparent Wilson's Disease will become seriously ill and eventually die if they are not treated.

Sometimes Wilson’s is diagnosed by the presence of the presence of Kayser-Fleischer rings, which are rusty-brown colored ring in the cornea. Diagnoses are usually made by blood and liver tests. Chelation therapy is commonly used as treatment. Doctors will also recommend avoiding foods high in copper such as liver, shellfish, mushrooms, nuts, chocolate, dried fruit, dried peas, beans and lentils, avocados, and bran.
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**Modificato dall'autore il 29.01.2020 11:32**
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