Wilsons Disease

Http: //www.wilsonsdisease.org/ Wilson's disease is a deadly inherited disorder unless copper poisoning is tested and handled before it causes serious illness. Wilson's disease affects 30,000 people worldwide. Genetic defects lead to excess copper accumulation. Small amounts of copper are essential as vitamins. Copper is included in most foods, and most people get more than necessary. A healthy person excrements unnecessary copper, but Wilson's patients can not take it.

Wilson's disease is an inherited disorder in which excessive copper accumulates in the body, especially the liver, brain and eyes. The signs and symptoms of Wilson's disease usually appear first between 6 and 45 years of age, but are most common in adolescence. Characteristics of this disease include a combination of liver disease and neurological and psychiatric problems. Liver disease is usually the first characteristic of Wilson's disease in sick children and young people; they may have very mild liver disease, but elderly people usually have symptoms of liver problems Is not ... Symptoms and signs of liver disease include yellowish white or white eyes (jaundice), fatigue, anorexia, swelling of the abdomen.

Wilson's disease is an autosomal recessive disorder due to a mutation in the Wilson disease protein (ATP7B) gene. For affected people, they must inherit copies of the affected genes from each parent. Diagnosis may be difficult, usually including blood tests, urinalysis, and liver biopsy. Genetic testing can be used to screen families of affected individuals. Wilson's disease is usually caused by changes in diet and medication. Changing the meal involves eating low copper diet without using copper cookware. Drugs used include chelating agents such as trientine and d-penicillamine, and zinc supplements. Complications of Wilson's disease include liver failure, liver cancer, and kidney problems. Liver transplantation may be useful for other patients with ineffective treatment or liver failure