2018年9月29日 · Wilson’s disease, also known as hepatolenticular degeneration and progressive lenticular degeneration, is a rare genetic disorder that causes copper poisoning in the body. Wilson’s disease...

Wilson's disease (also called hepatolenticular degeneration) is a genetic disorder characterized by the excess build-up of copper in the body. Symptoms are typically related to the brain and liver. Liver-related symptoms include vomiting, weakness, fluid build-up in the abdomen, swelling of the legs, yellowish skin, and itchiness.

2023年8月7日 · Wilson disease or hepatolenticular degeneration is an autosomal recessive disease which results in an excess copper build up in the body. It primarily affects the liver and basal ganglia of the brain, but it can affect other organ systems too. Symptoms usually are related to the brain and liver.

Learn how to diagnose and treat the causes and symptoms of Wilson's disease – or hepatolenticular degeneration – from the team of liver specialists at Bon Secours.

2019年1月3日 · Wilson Disease or Hepatolenticular Degeneration is a genetic pathological condition. It is an autosomal recessive disorder meaning that the affected individual needs to inherit the defective gene from both the parents.

Wilson disease (WD; also known as hepatolenticular degeneration) was first described in 1912 by Kinnear Wilson as “progressive lenticular degeneration,” a familial, lethal neurological disease accompanied by chronic liver disease leading to cirrhosis.

2025年1月20日 · Damage to brain tissue mainly occurs in an area called the lenticular nucleus. Hence, Wilson's disease is sometimes also called hepatolenticular degeneration. What causes Wilson's disease? In Wilson's disease, a particular gene on chromosome 13 does not work. The gene is called ATP7B.

Hepatolenticular degeneration is caused by mutations (changes) in the ATP7B gene. It is a genetic disorder that is inherited in an autosomal recessive manner and is usually diagnosed in teens and young adults. It is a type of copper storage disease. Also called Wilson disease.

deterioration; change from a higher to a lower form, especially change of tissue to a lower or less functionally active form. When there is chemical change of the tissue itself, it is true degeneration; when the change consists in the deposit of abnormal matter in the tissues, it …

2023年5月15日 · Wilson disease (WD) is caused by autosomal variants affecting the ATP7B gene on chromosome 13, resulting in alterations in physiological copper homeostasis and copper accumulation. Excess copper clinically manifests in many organs, most often in the central nervous system and liver, ultimately causing cirrhosis and death.