The new treatment for Wilson's disease is Cuvrior, a new trientine tetrahydrochloride (TETA-4HCl).
Penicillamine link (Cupramine, Depen) and trientine (Syprine) are two chelating agents used to treat Wilson disease.
These medicines remove copper from the body.
In Wilson's disease copper values over 100 μg will be found in urine.
Wilson's disease can go undiagnosed as it's a rare disorder with protean manifestations.
Because of this the diagnosis and treatment for Wilson's disease are delayed often.
Mutations in a manganese transporter, SLC30A10, produce hepatic cirrhosis due to manganese accumulation can be mistaken for Wilson's disease as it can mimic Wilson's disease.
The personality changes in Wilson's disease are aggression, Irritability, shallow cheerfulness, euphoria, social disinhibition, hypersexuality, lack of criticism and emotional lability.
When a person has Wilson's disease it leads to a build up of copper which damages certain structures such as the eyes, kidneys, brain, nervous system and liver and is fatal without medical treatment.
Wilson disease prevents your body from eliminating or getting rid of the copper in the body.
The neurological symptoms of Wilson's disease are ataxia, cognition difficulties, tremors, choreoathetosis, parkinsonism, dystonia and dysarthria.
The tests for Wilson's disease includes urine tests and blood tests, liver biopsy and genetic testing.
The blood tests can monitor the function of your liver and check the level of a protein called ceruloplasmin which binds copper in the blood.
The gold standard test for Wilson's disease is a liver biopsy test.
Wilson's disease is a rare genetic disorder involving the liver and brain, with onset frequently in adolescence.
Psychiatric symptoms are often the first manifestation of the disease and can obscure the diagnosis.
Wilson's disease is a disorder that is inherited which causes too much copper to accumulate in your organs.
With Wilson's disease the copper in the body is not eliminated properly and instead it accumulates or builds up and even to sometimes a life threatening level.
People who are most likely to get Wilson's disease are females and those between the ages of 5 to 40 and people that have a family history of Wilson disease.
Wilson's disease is triggered by an inherited defect in the ATP7B gene and is an autosomal recessive disorder which means that both parents must pass on the same abnormal gene to their child.
The gender that Wilson's disease is most common in is females as females are more likely to get Wilson disease than males.
With treatment you can live a full and healthy life with Wilson's disease.
Without treatment, Wilson disease is fatal.
The longer the copper poisoning continues, the harder it is to successfully treat, so early diagnosis is important.
The aims of treatment are to reduce the amount of copper in the body and control the symptoms.
Treatment for Wilson's disease must be lifelong.
The life expectancy for Wilson syndrome is up to 40 years if diagnosed by 12 to 23 years of age.
However with proper treatment and early enough diagnoses you can increase your life expectancy with Wilson syndrome.
Both parents have to have Wilson's disease for the child to get Wilson's disease.
If both the parents have the gene mutation for Wilson's disease then their child or children also have a 25 percent chance of getting Wilson's disease.
However if only one parent has the gene mutation for Wilson's disease then their child or children will not get Wilson disease although they may carry the gene mutation which they could then pass down to their children.
The age that Wilson's disease is usually diagnosed is between the ages of 5 years old to 35 years old although younger and older people can also be affected by Wilson's disease.
Copper in the body plays a very important role in building and maintaining healthy collagen and skin pigment melanin, bones and nerves.
The symptoms of Wilson's disease are hard time speaking, clumsy walking, involuntary muscle movements and tremors, abdominal pain, fatigue and swelling.
Wilson's disease is a disorder that is inherited which causes too much copper to accumulate in your organs.
With Wilson's disease the copper in the body is not eliminated properly and instead it accumulates or builds up and even to sometimes a life threatening level.
Symptoms of Wilson's disease most often begin between the ages of 12 to 23 years of age.
The symptoms of Wilson's disease include fatigue, abdominal pain, uncontrolled or poorly coordinated movements and swelling.
Treatment for Wilson's disease includes medications which can prompt your organs to release the copper into the bloodstream.
After the copper is released into your bloodstream it can then be eliminated and removed from your body through your kidneys.
Wilson disease is caused by an inherited defect in the ATP7B gene.
Wilson disease is an autosomal recessive disorder which means that both parents must pass on the same abnormal gene to the child.
Many times parents, who have only one abnormal gene will show no signs of the disease but are carriers of the disease.
There is no cure for Wilson's disease but Wilson disease can be managed.
Around 1 in 30,000 people have Wilson disease.
The Wilson disease is caused by faults in a gene called ATP7B, which must be inherited from both parents for the disease to be expressed.
Wilson disease is also known as hepatolenticular degeneration.
Usually, symptoms of Wilson's disease develop between 12 and 23 years of age, and untreated people may have a life expectancy of 40 years.
However, early diagnosis, followed by proper treatment, may increase the life span.