Wilsons disease- A brief medical study.

Wilsons disease- A brief medical study.

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  WILSONS DISEASE –ABRIEF MEDICAL STUDY PREPARED BY DR. MARTIN SHAJI
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   Wilsons diseaseis a genetic disorder, where there is too much copper in the body.  Small amounts of copper are needed in the body, but to much can be hazardous to the liver, brain, eyes, and other org...  Another name for Wilson's disease is Hepatolenticular degeneration.  The special proteins in the liver are called Hepacytes.  Degeneration of the liver and the central nervous system. A BRIEF DESCRIPTION
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  How It RelatedTo Bile Secretions… • Copper is absorbed from your food, and any excess is excreted through bile, required for the digestion of food, is excreted by the liver into passages In Wilson disease, copper does not pass from the ... Wilson's disease is named after Dr. Wilson, a doctor at the National Neurology Hospital in London. He discovered the disease in 1911
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  • Wilson's diseaseis caused by a mutation located on the 13th chromosome called ATP7B. • Mutations in the ATP7B gene produce a protein with decreased ability to bind copper. Inheritance • Autosomal recessive disease In order to inherit Wilson disease, both parents must carry one genetic mutation • Wilson disease carriers, who have only one copy of the abnormal gene, do not have symptoms
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  Symptoms  Wilson's diseasefirst attacks the liver, the  Central nervous system, or both.  Swelling of the liver or spleen  Jaundice  Fluid build-up in the legs or abdomen  Fatigue  A tendency to bruise easily
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  COMPLICATIONS
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   Chelating agentssuch as d-penicillamine and trientine. Other agents include sodium dimercaptosuccinate, dimercaptosuccinic acid, zinc, and tetrathiomolybdate. MANAGEMENT patients who are de-coppered and dependent to penicillamine. This medicine combines with excess copper in the body and may prevent your body from absorbing the copper in the foods you eat.(this is an exclusive drug for Wilson's disease)
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   The useof surgical decompression or trans jugular intrahepatic shunting (TIPS) in the treatment of portal hypertension is reserved for individuals with recurrent or uncontrolled variceal bleeding that is unresponsive to standard conservative measures.  Liver transplantation or prolong period or severe cases in accordance with the opinion of surgical gastroenterologist and hepatologist.  Healthy diet  Zinc salts (zinc acetate) act as inductors of metallothionein's, which favor a negative copper balance and a reduction of free plasmatic copper. must follow a diet that is low in copper. Foods to avoid include beef liver, cashews, black-eyed peas, vegetable juice, shellfish, mushrooms, and cocoa. People with this disease should not take any vitamin or mineral supplement that contains copper
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