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Wilson Disease

An inherited disorder in which too much copper builds up in the liver and is slowly released into other parts of the body. The overload can cause severe liver and brain damage if not treated with medication.

PubMed Health Glossary
(Source: NIH - National Institute of Diabetes and Digestive and Kidney Diseases)

About Wilson Disease

Wilson disease is a genetic disease that prevents the body from removing extra copper. The body needs a small amount of copper from food to stay healthy; however, too much copper is poisonous. Normally, the liver filters extra copper and releases it into bile. Bile is a fluid made by the liver that carries toxins and wastes out of the body through the gastrointestinal tract. In Wilson disease, the liver does not filter copper correctly and copper builds up in the liver, brain, eyes, and other organs. Over time, high copper levels can cause life-threatening organ damage.

What is the liver?

The liver is the body's largest internal organ. The liver is called the body's metabolic factory because of the important role it plays in metabolism—the way cells change food into energy after food is digested and absorbed into the blood. The liver has many important functions, including...Read more about Wilson Disease NIH - National Institute of Diabetes and Digestive and Kidney Diseases

What works? Research summarized

Evidence reviews

Systematic review: clinical efficacy of chelator agents and zinc in the initial treatment of Wilson disease

This review of chelator agents and zinc for initial treatment of Wilson disease concluded that there was a lack of high-quality evidence for the comparative efficacy of different drugs. Based on the low quality of the evidence this conclusion is reliable, but more information on the review methods is needed to confirm the reliability of the review.

Clinical efficacy and safety of Chinese herbal medicine for Wilson's disease: a systematic review of 9 randomized controlled trials

Wilson's disease is an autosomal recessive disorder of copper metabolism. Despite being treatable, there is no universally accepted treatment regimen. Currently, various Chinese herbal medicines (CHMs) are widely used in the treatment of Wilson's disease in China, but there is a lack of reliable scientific evidence for the effectiveness of such therapies. The objective of this systematic review is to assess the clinical efficacy and safety of CHM as an alternative or/and adjuvant therapy for Wilson's disease. A systematic literature search in different medical databases was performed to identify randomized controlled trials comparing CHM as monotherapy or CHM as adjuvant therapy with western conventional medical therapy in the treatment of Wilson's disease. A total of 687 participants were included in nine eligible studies. The main findings are that CHM as monotherapy or adjuvant therapy for Wilson's disease may be able to improve the clinical symptoms, to promote the urinary copper excretion, to ameliorate liver function and/or liver cirrhosis, and has fewer adverse effects in comparison with western conventional medication. Furthermore, CHM generally appeared to be safe and well tolerated in patients with Wilson's disease. However, the evidence presented in this review are insufficient to warrant a clinical recommendation due to the generally low methodological quality of the included studies. In conclusion, CHM seems to be beneficial and safe for Wilson's disease, but high-quality evidences are still needed to further evaluate this therapy. Therefore, additional well-designed, randomized, placebo-controlled clinical trials are needed.

Alcohol Use Disorders: Diagnosis and Clinical Management of Alcohol-Related Physical Complications [Internet]

Alcohol is the most widely used psychotropic drug in the industrialised world; it has been used for thousands of years as a social lubricant and anxiolytic. In the UK, it is estimated that 24% of adult men and 13% of adult women drink in a hazardous or harmful way. Levels of hazardous and harmful drinking are lowest in the central and eastern regions of England (21–24% of men and 10–14% of women). They are highest in the north (26–28% of men, 16–18% of women). Hazardous and harmful drinking are commonly encountered amongst hospital attendees; 12% of emergency department attendances are directly related to alcohol whilst 20% of patients admitted to hospital for illnesses unrelated to alcohol are drinking at potentially hazardous levels. Continued hazardous and harmful drinking can result in dependence and tolerance with the consequence that an abrupt reduction in intake might result in development of a withdrawal syndrome. In addition, persistent drinking at hazardous and harmful levels can also result in damage to almost every organ or system of the body. Alcohol-attributable conditions include liver damage, pancreatitis and the Wernicke’s encephalopathy. Key areas in the investigation and management of these conditions are covered in this guideline.

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Summaries for consumers

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Expert-reviewed information summary about the causes and management of nutritional problems that occur in patients with cancer.

Terms to know

Fluid made by the liver and stored in the gallbladder that helps break down fats and get rid of wastes in the body.
Blood Tests
Blood tests help doctors check for certain diseases and conditions. They also help check the function of your organs and show how well treatments are working.
The part of the central nervous system that is contained within the skull (cranium).
Imaging Tests
A type of test that makes pictures of areas inside the body. Some examples of imaging tests are CT scans and MRIs. Also called imaging procedure.
One of a pair of organs in the abdomen. The kidneys remove waste and extra water from the blood (as urine) and help keep chemicals (such as sodium, potassium, and calcium) balanced in the body. The kidneys also make hormones that help control blood pressure and stimulate bone marrow to make red blood cells.
The largest abdominal organ. The liver carries out many important functions, such as making important blood proteins and bile, changing food into energy, and cleaning alcohol and poisons from the blood.
Metabolic Disorders
A condition in which normal metabolic processes are disrupted, usually because of a missing enzyme.

More about Wilson Disease

Photo of a young adult

Also called: Hepatolenticular degeneration, Wilson's disease

Other terms to know: See all 7
Bile, Blood Tests, Brain

Keep up with systematic reviews on Wilson Disease:


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