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Wilsons disease

Wilson disease (also known as hepatolenticular degeneration) is a genetic disorder thatprevents the body from getting rid of extra copper.

A small amount of copper obtained from food is needed to stay healthy, but too much

copper is poisonous. In Wilson disease, copper builds up in the liver, brain, eyes, and

other organs. Over time, high copper levels can cause life-threatening organ damage.

Causes

Wilson disease is caused by a buildup of copper in the body.

Normally, copper from the diet is filtered out by the liver and released into bile, which

flows out of the body through the gastrointestinal tract. People who have Wilson disease

cannot release copper from the liver at a normal rate, due to a mutation of the ATP7Bgene. When the copper storage capacity of the liver is exceeded, copper is released intothe bloodstream and travels to other organsincluding the brain, kidneys, and eyes.

Hepatic fibrosis develops, ultimately producing cirrhosis. Copper diffuses out of the liver

into the blood, then into other tissues. It is most destructive to the brain but also damages

the kidneys and reproductive organs and causes hemolytic anemia. Some copper isdeposited in Descemet's membrane of the cornea, causing Kayser-Fleischer rings.

Symptoms

Wilson disease first attacks the liver, the central nervous system, or both.

A buildup of copper in the liver may cause ongoing liver disease. Rarely, acute liver

failure occurs; most patients develop signs and symptoms that accompany chronic liver

disease, including

swelling of the liver or spleen

jaundice, or yellowing of the skin and whites of the eyes

fluid buildup in the legs or abdomen

a tendency to bruise easily

fatigue

Kayser-Fleischer rings result from a buildup of copper in the eyes and are the most

unique sign of Wilson disease. They appear in each eye as a rusty-brown ring around the

edge of the iris and in the rim of the cornea.

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A buildup of copper in the central nervous system may result in neurologic symptoms,

including

problems with speech, swallowing, or physical coordination

tremors or uncontrolled movements

muscle stiffness behavioral changes

Motor deficits are common, including any combination of tremors, dystonia, dysarthria,

dysphagia, chorea, drooling, and incoordination.

Diagnosis

Wilson disease is diagnosed through a physical examination and laboratory tests.

In case of physical examination, Kayser-Fleischer rings are present in almost all people

with Wilson disease who show signs of neurologic damage but are present in only 50percent of those with signs of liver damage alone.

Laboratory tests measure the amount of copper in the blood, urine, and liver tissue.

Most people with Wilson disease will have a lower than normal level of copper in theblood and a lower level of corresponding ceruloplasmin, a protein that carries copper in

the bloodstream. In cases of acute liver failure caused by Wilson disease, the level of

blood copper is often higher than normal. A 24-hour urine collection will show increasedcopper in the urine in most patients who display symptoms.

A liver biopsya procedure that removes a small piece of liver tissuecan show if theliver is retaining too much copper.

Genetic testing may help diagnose Wilson disease in some people, particularly those with

a family history of the disease.

In Ayurvedic perspective

Drugs that possess properties such as rakta prasadana and yakrit visesha should be

selected.

Tikta rasa

ShophaghnaMutrala

Vishahara

The following medicines can be given in this case

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Kashayam and Paneeyam

Guduchyadi Kashayam 60 ml twice daily before food (A.H)

Punarnavadi kashayam 60 ml twice daily before food (Bhaishajya

Ratnavali)

Nimbatvagadi kashayam 60 ml twice daily before food (S.Y) Patolakaturohinyadi kashayam 60 ml twice daily before food

Laghu Panchamoola Paneeyam 500 ml daily

Jambeeradi Paneeyam 20 ml twice daily

Tablets

Dashanga Agadam 2 tablets twice daily after food

Dushi Vishadi Agadam 2 tablets twice daily after food

Chandraprabha vati 2 tablets twice daily after food

Lehyam

Drakshadi Lehyam 1 teaspoon twice daily after food

Dashamoola harithaki 10 grams at night after food (A.H)

Rasayanam

Tippili Rasayanam

Choornam

Pippili choornam 1 teaspoon twice daily with honey after food

Bhrungaraja Choornam 1 teaspoon twice daily with honey after food

Neeli Choornam 1 teaspoon twice daily with honey after food

Nimba Tvak Choornam 1 teaspoon twice daily with honey after food

Guda Pippali 1 teaspoon twice daily with milk after food

External application

In case of tremors, Sahacharadi tailam can be used for external application

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