Brain Wilson

The disease of Wilson is a genetic disorder, that is fatal unless early detected and treated. The this is a rare condition, where the additional copper develops in the body causing to poison it copper. The disease of Wilson is completely rare and affects 1 in 30,000 people. It is after named Dr Samual Wilson, that described first the disorder in 1912.

The copper is a 'the track metal,' that is found in a lot of foods. Some quantity of copper take is demanded to remain necessarily healthy. Normally, the body itself dedbarasse automatically of the additional copper, but a person suffering from the disease of Wilson is not capable naturally to excrete the additional copper. This has for result gradual deposition of the additional copper in the vital organs as the liver, the brain, the cornea and the kidneys ae" and so done not treat on time, can reveal itself potentially fatal.

Generally, the first party of the body to be affected by the copper deposits is the liver. In half of the Wilson disease to suffer the patients, the liver is the only affected organ. Too much copper in the liver cells (the hepatocytes) is harmful and takes to the liver damages.

The additional copper damages also the fabric of brain in a sector called the kernel of lenticular. Therefore, the disease of Wilson also is called 'the hepato-lenticular Degradation'. If left non-treaty, the damages can become harsh and finally fatal.

Which is the cause of disease of Wilson?

The basic basic lack in the disease of Wilson is not known. It could be related to the inability of the body to produce sufficient levels of ceruloplasmin, an enzyme in the fluid portion of the blood that links to the copper and is implied in his transportation and his regulation. The scientific others believe that the reduced production of ceruloplasmin can be the result of a defect in the liver capacity to break down of the copper. The correlation between the levels of ceruloplasmin reduces and the accumulation of excessive copper completely is not understood. Besides, there is also proof of reduced excretion of copper by the system of biliary.

How does the one obtain the Disease of Wilson?

The disease of Wilson is the disease rather rare. It genetically especially is related; therefore, most of the people inherit it of their parents. The this is also completely possible for the mutation to arrive spontanedment. An outside 40,000 people inherits the disease of Wilson of their parents, and 1/120,000 (an outside four victims of disease of Wilson) obtains the disease of a spontaneous mutation.

What the problems are they related to the disease of Wilson?

Although the genetic defect already is present to the birth, it takes years for the additional copper to develop at the toxic level where it damages inevitably.

The disease symptoms typically begin developing between the ages of 6 and 20, the most ordinarily in the adolescent years. Nevertheless, you can develop also symptoms in the average age.

The sign the more characteristic of this disease is the kayser-fleischer ring - a brown ring rusty around the eye cornea that only can be seen by an eye examination. The others sign depend on if the damages arrive in the liver, the blood, the central nervous system, the urinary system, or the system squeletto-musculaire.

1. Liver problems ae"

The symptoms of disorders of liver are often the first one to develop. The toxic effect on the liver cells can cause the hepatitis (the liver inflammation) taking jaundice, the pain and the abdominal vomitings. If left non-treaty, the damages to the liver cells can cause to mark liver (cirrhosis). Finally harsh cirrhosis and liver failure develop in the non treated case causing harsh problems.

2. Brain problems ae"

As the copper obtains deposited in the brain, it can cause various symptoms:

aec The Physical symptoms as a curious type of trembling in the arms, the movement slowness, the difficulty with the speech (dysphagia), writing problems, the downhill of difficulty, an aspen the gait, the headaches, the seizures.

aec The Psychological symptoms as the depression, the mood swings, the strange and inopportune behavior, the thought depression, neurosis, or the suicidal psychosis accompanied of, the inability to concentrate. The affected people can become very aggressive and sentimental; and can expose a 'the change in the personality'.

If the disease of not Wilson left treated, the copper accumulation in the brain can take to the harsh problems as muscular harsh weakness, the stiffness in the body, and insanity.

3. Other problems ae"

The copper can develop in the eye cornea. This causes a characteristic characteristic called the kayser-fleischer beeps - a brownish pigmentation of the cornea. The characteristic others that can develop include weakens it, the kidney damages, the heart problems, pancreatitis (the pancreas inflammation), the problems of menses and the miscarriage repeated in the women, and premature osteoporosis (diluting bones).

The disease of Wilson is diagnosed with the tests that measure the quantity of copper in the blood, the urine, and the liver.



How is the disease of Wilson diagnosed?

If the disease of Wilson is suspected, it is diagnosed by the tests as -

aec A blood analysis to measure ceruloplasmin - a protein that secures copper in the sanguine current.

aec A urine test to measure the quantity of copper in the urine.

aec A cornea examination can show the kayser-fleischer beeps themselves they developed.

aec A liver biopsy

If the disease of Wilson obtains confirmed in a person, a then brothers and a sisters also should be tried to see themselves they have the condition. The brothers or sisters of a person with the disease of Wilson have a 1/4 chance to have the condition.

Is there a remedy for the Disease of Wilson?

The remedy for the disease of Wilson implies a liver transplantation, that is executed if necessary to save the life of the person. The person will always have the genetic defect, and initial damages to the nervous system will not go, but can improve.

How is the disease of Wilson treated?

The objective of therapy of drug in the individuals with the disease of Wilson is to remove additional copper of the body and prevents from the accumulation of continuous copper and deposition. Therefore, drug therapy must be continued through life. The preceding treatment is begun, the better the chance to prevent from the permanent damages in the long term to the liver or to the brain.