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Wilson's disease [wil´sunz]
a rare progressive disease, inherited as an autosomal recessive trait and due to a defect in metabolism of copper
, with accumulation of copper in the liver, brain, kidney, cornea, and other tissues. Characteristics include cirrhosis of the liver and degenerative changes in the brain, particularly the basal ganglia. Liver disease is the most likely manifestation in children; neurologic disease is most common in young adults. The characteristic ophthalmic feature is a pigmented ring (Kayser-Fleischer ring
) at the outer margin of the cornea. Called also hepatolenticular degeneration
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.
Wilson's disease Neurology An AR disorder of copper metabolism characterized by accumulation of copper in the brain, liver, and other organs Clinical Cirrhosis, degeneration of basal ganglia and neurologic deterioration with involuntary movement, tremors, muscular rigidity, spastic contractures, psychiatric defects, dysphagia, Kayser-Fleischer ring. See Ceruloplasmin, Copper.
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.
Wilson's disease A rare genetic disorder in which copper accumulates in the body, especially in the liver and brain, causing CIRRHOSIS and brain damage. There are behaviour abnormalities and personality changes, writhing movements of the limbs (athetoid movements), muscle rigidity and shortening, dementia and, in untreated cases, death. If diagnosed early, often by the observation of a ring of greenish-brown discoloration in each cornea (KAYSER-FLEISCHER RING), the condition may be controlled with a diet low in copper and the use of the drug d-penicillamine, which binds copper into a form which is excreted in the urine. Also known as hepato-lenticular degeneration. (Samuel Alexander Kinnier Wilson, 1878–1937, American-born English neurologist).
Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005
An inborn defect of copper metabolism in which free copper may be deposited in a variety of areas of the body. Deposits in the brain can cause tremor and other symptoms of Parkinson's disease.
Gale Encyclopedia of Medicine. Copyright 2008 The Gale Group, Inc. All rights reserved.
Patient discussion about Wilson's disease
Q. Hi this is Wilson; I have a daughter, who is 5. She has frequent nose bleeds when she is behaving badly. Hi this is Bell; I have a daughter, who is 5. She has frequent nose bleeds when she is behaving badly. She has been screened by her school for ADHD and I haven’t had a chance to consult a doctor for her screening and exam for ADHD. I thought that her nose bleeds cause due to her behavior? I need help.
A. I don’t have an idea about this. But I think that you should first discuss these issues with your doctor. Also mention to the doctor if she has any problems with sleeping, like only sleeping 6 hours a night. Try with occupational Therapists which help children and adults with small motor issues like handwriting and also with sensory issues like learning how to calm down. Early intervention is always best.More discussions about Wilson's disease
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