Wilson's Disease

Wilson’s Disease Symptoms And Treatment

Wilson’s Disease Symptoms And Treatment is our today topic.The two eyes above belong to the same person. A photograph shows a kayser-fleischer ring due to Wilson’s disease. The second photo was taken after drug use.

Wilson’s disease is a genetic disorder caused by the accumulation of copper in the body, especially in the liver and brain. Wilson’s disease can cause very serious symptoms if not treated. Treatment is aimed at removing copper from excess copper by some medicines.

Wilson’s Disease Symptoms And Treatment

What is Wilson’s Disease?
Wilson’s disease is caused by the accumulation of copper in the body and is a very rare hereditary disease in 1 out of every 30,000 people. Dr. Wilson first described the disease in 1912. It comes from Samuel Wilson.

Copper, which is the main cause of the disease, is a trace metal found in many foods. We need very little to look at to remain healthy. Normally, the body needs more surplus body wounds, but Wilson patients can not throw away this copper body and copper accumulates especially in the liver, brain, cornea and kidneys.

The excess copper in the liver cells causes damage to the liver. Damage to the brain cells occurs primarily in a region called the lenticular nucleus.

The cause of Wilson’s disease
In Wilson’s disease, a specific gene called ATP7B on chromosome 13 is inoperable. This gene normally controls the liver cells’ excess pulse. Liver cells normally send excess copper bile to the liver. When this process is carried out, copper accumulates in the liver cells. When the copper storage capacity of the liver is too much, it is confused with copper and accumulates in different parts of the body, mainly the brain.

Wilson’s Disease Symptoms And Treatment

Wilson’s Symptoms
In Wilson’s patients, the genetic disorder is born, but it takes years for copper to reach health-damaging dimensions in the body. The symptoms usually start to appear between the ages of 6 and 20 years.

1. Liver Problems

First, the liver indicator appears. The toxic effect of liver cells is caused by hepatitis (liver inflammation), which can cause cramps, vomiting and vomiting in the abdomen. If the disease is left untreated, cirrhosis may also lead to liver failure, leading to cirrhosis.

2. Brain Problems

Coppersmith can lead to different expressions as they accumulate:

It causes physical symptoms such as trembling, slowness in movements, difficulty speaking, writing problems, difficulty swallowing, walking swaying, headaches and illnesses.
Psychological symptoms such as depression, turbulent moods, impaired concentration may also occur.
If untreated, accumulation of copper in the brain leads to weakness, stretching difficulties and perspiration in the muscles.

Wilson’s Disease Symptoms And Treatment

3. Other Problems

Copper may also accumulate in the cornea in the eye. This leads to a brownish pigmentation called the kayser-fleischer ring in the cornea.
Other symptoms that may be seen are anemia, kidney damage, heart problems, pancreatitis (pancreatitis), menstrual problems in women, abortion and osteoporosis in premature birth (bone erosion).

Diagnosis of the disease
If Wilson’s disease is suspected, several tests can be used to diagnose it:

A blood test is performed to measure the ceruloplasmin. Seruloplazmin is a protein that keeps on looking. In Wilson’s disease, the amount of this protein is very low.
Other blood tests are done to check the copper level, kidney and liver function.
A urine test is performed to measure the amount of copper in the head. This test is usually done by checking all the urine produced for 24 hours. The amount of copper in the head is usually above normal.

If the test is made on the cornea by the eye specialist, it also reveals the kayser-fleischer ring formation.
A sample taken from the liver is examined under a microscope. This is called a biopsy. This reveals the excess of copper in the liver and the size of the cirrhosis.
Experts can also perform brain tomography and kidney screening tests
If the patient is diagnosed with Wilson’s disease, the siblings of the patient should be checked with the possibility of having the same disease. The likelihood that a sibling of a Wilson patient is also sick is 1 in 4.

Treatment of Wilson’s Disease
The sooner the disease is started to treat, the more likely it is to prevent the problems that may occur in the liver and the brain in the long term.

Penicillamine
is a kind of needle that is used to throw excess copper in the body. Penicillamine helps to remove excess copper from the body through the urine. After the build-up of copper is overcome, the syringe is lowered in 1 year.

Trientine
Penisalimine is an alternative needle. This needle acts as a clamping effect, allowing the copper to excrete from the body.

Wilson’s Disease Symptoms And Treatment

Zinc
In some cases it is another alternative. Zinc blocks the absorption of copper from the intestines’ nutrients. Thus, even if zinc is not thrown away with too much copper, it has prevented copper accumulation. Zinc treatment is less likely to cause side effects than penicillamine and trientine. Zinc therapy may be an option in the first phase when Wilson’s disease has not yet begun to show symptoms. In addition, zinc therapy can be used in patients whose copper levels have been reduced to reasonable levels initially by treatment with penicillamine and trientine. Zinc can also be taken in pregnancies.

Note : The treatment of Wilson’s disease is a lifelong process. At first, a lot of copper is thrown out of the body. Then the accumulation of copper is prevented. If negligence is made in taking medicines, copper accumulation may start again and result in lethal events.

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