Wilson disease is also called hepatolenticular disorder. It is an autosomal recessive genetic disorder in which there is an accumulation of copper in the tissues. Wilson disease involves neurological symptoms and liver disease.
Copper is needed by the body for the development of healthy, bones, nerves, collagen and melanin. Copper is obtained by food in a small amount that is needed by the body, but too much of it is bad. The liver takes up the needed amount of copper and rest of it is excreted in bile. In a person affected with Wilson disease, the excess copper is not eliminated and it gets deposited in brain, eyes, liver and other organs. With time it produces damage.CES life-threatening organ
In Wilson, disease body is prevented from getting rid of extra copper. The disease first attacks the liver, central nervous system or both.
A characteristic diagnostic sign of Wilson disease is a formation of Kayser-Fleischer Ring. It is a brownish yellow ring that is visible at the corneoscleral junction. There is a copper deposition in Descemet’s membrane that extends to the trabecular meshwork.
There are four stages in the Wilson disease:
• Stage I: The copper is accumulated within hepatic binding sites.
• Stage II: There is the acute redistribution of copper in the liver and it is released into the circulation.
• Stage III: Copper is accumulated in the brain and other extra-hepatic tissues.
• Stage IV: Copper balance is restored by the use of long-term chelation therapy.Age / Sex prevalance of Wilson Disease
Symptoms of Wilson disease appears between 6 to 20 years of age.
Men and women are affected equally.
Normally the copper that is obtained from the diet is filtered by the liver and released into the bile. But in a person having Wilson disease copper is not filtered from the liver because of the mutation of the gene ATP7B. Copper starts getting accumulating in the liver and after the capacity of a liver is exceeded the copper is released into the bloodstream, it then travels to other organs.
The genetic mutation commonly passes from one generation to other. The genetic mutation that is responsible for the development of Wilson disease results in problems with a protein that s needed for removing excess of copper out of the liver.
Copper is needed by the body for the development of healthy, bones, nerves, collagen and melanin.
• Swelling of liver or spleen
• Easy bruising
• Swelling of arms and legs
• Behavioral changes
• Muscle stiffness
• Problems in physical coordination, speech or swallowing
• Osteoporosis, arthritis
• Low platelet or WBC count
• Raised level of uric acid, amino acid, protein and carbohydrate
• A presence of Kayser-Fleischer Ring.
Diagnosis of Wilson disease is done through physical examination and lab test.
• Blood and urine test
• CT and MRI of brain
• Examination of eye under slit lamp
• Liver biopsy
• DNA mutation analysis
Medicines are used in order to reduce the amount of copper in the body. After the copper level is reduced treatment aims at preventing the copper from accumulating again.
A liver transplant may be needed in case if the liver damage is irreversible.
• Acute liver failure
Food to be avoided that contains high amount of copper:
Shellfish, squid, pork, lamb, salmon, tofu, mushroom, dry fruits, millet, lentils, barley, soy products, wheat germ, bran bread, fresh sweet potatoes, cocoa, mineral water.Alcohol should be avoided to lessen further damage to the liver.
Food to be consumed that are low in copper:
Beef, chicken, turkey, Vegetables, Butter, mayonnaise, rice, bread and pasta of refined flour, cheese, tea, fruit juices.
• Wilson disease proves fatal without proper treatment.
• Life-long treatment is required in Wilson disease.
• Early detection and proper treatment give the affected person a healthy life and better chances of survival.
• Death occurs due to blood, liver or kidney problems.
• Liver cirrhosis
• Liver cancer
• Liver failure
• Kidney stones
• Neurological problem
• Main action on sympathetic nerves
• Tones ups appetite and digestion
• General feeling of well-being
• It is a vasomotor stimulant, main action on pneumogastric nerve
• Suppressed discharges
• A state of depression and relaxed condition
• Confused and depressed state
• Lymphatic inflammation
• Tendency to malignancy in acute and subacute disorders
• Hysteria , nervous affections
• Over sensitiveness, night hallucinations
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