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Wilson's disease is an inherited disorder that causes too much copper to accumulate in your liver, brain and other vital organs. Another term for Wilson's disease is hepatolenticular degeneration. Copper plays a key role in the development of healthy nerves, bones, collagen and the skin pigment melanin. Normally, copper is absorbed from your food, and any excess is excreted through bile — a substance produced in your liver. But in people with Wilson's disease, copper isn't eliminated properly and instead accumulates, possibly to a life-threatening level. Left untreated, Wilson's disease is fatal. When diagnosed early, Wilson's disease is treatable, and many people with the disorder live normal lives.
Complications of Wilson Disease
Wilson's disease can cause serious complications such as:
Causes of Wilson Disease
Wilson's disease occurs when a genetic mutation leads to an accumulation of copper in your body.
How the genetic mutation occurs
The genetic mutation that causes Wilson's disease is most commonly passed from one generation to the next. Wilson's disease is inherited as an autosomal recessive trait, which means that to develop the disease you must inherit two copies of the defective gene, one from each parent. If you receive only one abnormal gene, you won't become ill yourself, but you're considered a carrier and can pass the gene to your children.
How the genetic mutation causes Wilson's disease
The mutation that causes Wilson's disease occurs in a gene called ATP7B. When a mutation occurs on this gene, it leads to problems with a protein that's responsible for moving excess copper out of your liver. Your body collects copper from the food you eat during the digestive process. The copper is transported to your liver where liver cells use it for everyday tasks. Most people eat more copper than they need. In these cases, the liver takes what it needs and excretes the rest in bile, a digestive juice made by the liver. But in people with Wilson's disease, the extra copper doesn't leave your body. Instead, copper builds up in the liver, where it can cause serious and sometimes irreversible damage. In time, excess copper leaves the liver and begins accumulating in and harming other organs, especially the brain, eyes and kidneys.
Signs & Symptoms of Wilson Disease
Wilson's disease causes a wide variety of signs and symptoms that are often mistaken for other diseases and conditions. Signs and symptoms vary depending on what parts of your body are affected by Wilson's disease.
Signs and symptoms of Wilson's disease include:
Diagnosis of Wilson Disease
Diagnosing Wilson's disease can be challenging because its signs and symptoms are often indistinguishable from those of other liver disease, such as hepatitis. What's more, many symptoms may evolve over time, rather than appear all at once. Behavioral changes that come on gradually can be especially hard to link to Wilson's. Doctors rely on a combination of symptoms and test results to make the diagnosis.
Test and procedures used to diagnose Wilson's disease include:
Treatments of Wilson Disease
If you've been diagnosed with Wilson's disease, your doctor may recommend medications to reduce the amount of copper in your body. Once that is achieved, treatment focuses on preventing copper from building up again. When liver damage is severe, a liver transplant may be necessary.
Medications that remove excess copper from your body
Medications called chelating agents prompt your organs to release copper into your bloodstream. The copper is then filtered by your kidneys and released into your urine. Treatment for people with signs and symptoms of Wilson's disease usually begins with a chelating agent. Once your signs and symptoms are under control, your doctor may recommend a lower dose of medication to maintain a safe level of copper in your body. Doctors sometimes also recommend chelating agents to people who've been diagnosed with Wilson's disease, but don't have signs and symptoms. For these people, a chelating agent can reduce the risk of liver damage.
Side effects of chelating agents depend on the specific medication:
Medication to maintain healthy copper levels
Zinc acetate prevents your body from absorbing copper from the food you eat. Zinc is sometimes used in people who've had successful treatment with a chelating agent. In these people, zinc may help maintain a healthy copper level. Zinc is also used in people who've been diagnosed with Wilson's disease, but don't have any signs or symptoms. Zinc acetate causes few side effects, but can cause upset stomach.
For people with severe liver damage, a liver transplant may be necessary. During a liver transplant, a surgeon removes your diseased liver and replaces it with a healthy liver from a donor. Most transplanted livers come from donors who have died. But in some cases a liver can come from a living donor, such as a family member. In that case, the surgeon removes your diseased liver and replaces it with a portion of your family member's liver.
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