Wilson’s disease

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Wilson’s disease is also called hepatolenticular degeneration and progressive lenticular degeneration. It is a rare inherited disorder that causes the accumulation of copper on your brain, liver and other vital organs, leading to copper poisoning. Copper is helpful in the development of bones, nerves, collagen and melanin. Some amount of copper is absorbed by the food, rest is excreted from the system. A small amount of copper is needed to keep the body healthy, however excess of copper is poisonous. It affects about 1 in 30,000 people worldwide and is diagnosed among people from 5 to 35 years of age. However it can affect younger as well as older age groups.

The disease got its name from the Britain based Neuro-physician Samuel Alexander Kinnier Wilson, who described the condition in the year of 1912, along with its pathological impact on brain and Liver.

 

 

 

 

 

Causes:

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  • Wilson’s disease is an autosomal recessive trait, i.e. to develop the disease you must inherit one copy of the defective gene from each parent. If the patient receive an abnormal gene then he or she will not get ill to himself or herself, but he or she will be a carrier to pass that gene to the schildren.

 

 

 

 

 

 

 

 

Symptoms:

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  • Kayser-Fleischer rings, a golden brown discolouration of the eye.
  • Sunflower cataracts, a distinctive multi-coloured center with spokes radiating outwards.
  • Jaundice or yellowing of eyes.
  • Impairment of memory, speech and vision.
  • Problems with physical coordination.
  • Discolouration in nails.
  • Mood swings.
  • Changes in personality.
  • Irregularities in menstruation.
  • Premature osteoporosis.
  • Kidney stones.
  • Low blood pressure.
  • Abdominal pain.
  • Lack of appetite.
  • Fatigue

 

 

Diagnosis:

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  • Blood test: (Copper testing) Doctors recommend blood test to check the level of a protein that binds copper in the blood, ceruplasmin and the level of copper in blood.
  • Urine test: Doctors recommend urine test to measure the amount of copper in blood.
  • Eye exam: Doctors recommend an eye test to check the level of copper in eyes. This test is recommended for checking for conditions like Kayser-Fleischer rings and sunflower cataract.
  • Genetic testing: A blood test is conducted to identify mutations that can lead to Wilson’s disease.
  • Liver biopsy: Doctors recommend a biopsy, which involves extracting the liver tissue and examining it.

 

 

 

Treatment:

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  • Medications like Penicillamine (Cuprimine, Depen), Trientine (Syprine) and Zinc acetate (Galzin).

 

Surgery:

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Liver transplant is required when the liver damage is irreversible. This involves the transfer of a healthy liver from the body of the donor, into the body of the recipient.

Life after surgery:

Wilson’s disease is also called hepatolenticular degeneration and progressive lenticular degeneration.

Liver transplants are usually a great success. According to the most recent data(2004) one year survival rate post-transplant is approximately 86 percent for all liver transplant patients. About 78 percent of patients live upto 3 years after liver transplant, 72 percent of patients live upto 5 years after the surgery and 53 percent of patients live upto 20 years after the surgery.

After a liver transplant, a patient is recommended to stay in the I.C.U for a few days and then later shifted to the transplant recovery area for 5-10 days. The patient can then go back to normal day-to-day activities. Regular follow-ups are advised and patients have to take the medicines for lifetime.

 

Risks involved:

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Risks involved in a liver transplant include:

  • Organ rejection.
  • Failure of the donated liver.
  • Clots of blood.
  • Bile duct leaks.
  • Shrinking of bile ducts.
  • Long-term complications may also include recurrence of liver disease in the transplanted liver.

 

Prognosis:

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The earlier you find out if you have the gene for Wilson's disease, the better your prognosis is. Wilson's disease can develop into liver failure and brain damage if left untreated. Early treatment can help reverse neurological issues and liver damage.

 

 

 

 

Cost:

Wilson Disease Cost

The cost of Syprine is $200 per 250-mg capsule and Cuprimine costs$55-$60 per 250-mg tablet.1 The annual cost for the average daily adult dose of Syprine (1,000 mg) is $300,000, making it the most costly treatment for any liver disease to date.

In the late 90s, when the first successful liver transplants were done in India, the procedure cost Rs.20 lakh. Now, a resection costs between Rs.3 and 4 lakh and a transplant Rs.20-25 lakh in a private hospital.

 

Liver Transplant India offers a low-risk and most affordable way to treat Wilson’s disease. Our team of experienced and skilled doctors make life at hospital a painless one and guarantee a speedy recovery.

 

Wilson’s disease Doctors

 

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