how serious is wilson's disease?

Question

What is von willebrand s disease and how serious?

Answer 1

Life threatening : As a chronic metabolic disorder, wilson's is a rotten illness.If detected early and responsive to the limited treatments available, some semlance of a life is possible. Late DX and poor response leads to liver disease in childhood and neurologic degenerative effects in older patients. The kidneys and bones can also be injured.

Answer 2

Very: Wilson's disease can cause problems in liver, eyes, kidneys, and heart. It can also cause neuropsychiatric problems and problems with a patient's hormones.

Answer 3

Lethal: Neglect it, and it will kill you. It's a gruesome death, with brain damage (especially inability to move) compounded with liver failure.

Answer 4

Deg.Hepa Lenticular : Hepato-lenticulardegeneration. Diagnosis often missed; should be considered in patients aged 10 to 40 years with hepatitis, cirrhosis, hepatic decompensation, and symptoms suggestive of movement or psychiatric :can vary from loss of control of emotions to depression, delusions, loss of memory, inability to focus on tasks, impulsiveness, sexual disinhibition, and other bizarre behaviors.

Answer 5

Wilson Disease: The main organs affected by copper accumulation are the liver and brain, so your symptoms are refer able to these organs. You may experience cognitive impairment, bradykinesia, parkinsonian-like motor symptoms, other motor and sensory problems, fatigue, esophageal varices, liver and kidney disease, and, of course, the copper colored rings in your eyes. Cardiac involvement can also intervene.

Answer 6

Nonspecific: The mood changes, elevated liver enzymes, elevated reticulocyte count, and perhaps glucose or amino acids in the urine are all nonspecific. The ring round the cornea and the movement disorers are seen with advancing disease.

Answer 7

Copper: 1st get a copper level checked. Also ask for some liver function testing. They have a classic kaiser-fleischer ring around the iris of the eye....And lastly is to get a liver biopsy.

Answer 8

Old: Wilson disease may have partial inheritance, incomplete penetrance, and variation in the genetics of the disease, so that the only way a patient knows they have it, is when they are tested (often during work-up of a relative). Therefore, there is not really an age limit. However, most come to attention in early twenties to thirties.

Answer 9

From my memory: I recall a case from a patient who was around 60. Late-onset Wilson's is not unknown. There's a Slavic gene that tends to present late. http://www.ncbi.nlm.nih.gov/pubmed/17433323

Answer 10

It Alters Metabolism: In wilsons disease, genetic information to make a special protein is missing. That protein is supposed to carry excess copper like a taxicab.It takes it to the kidneys to be eliminated. Without this carrier protein, copper builds up in toxic amounts in tissue & starts to kill cells by messing up their metabolism. We are exposed to copper in our diet & in low levels it won't hurt you, high levels do.

Answer 11

Wilson's disease: Wilson's disease is caused by an abnormality in the way the body handles copper which leads to accumulation and toxicity.

Answer 12

Old term: This indicates that some component of the machinery that carries out the basic biology of the cells is defective. It can apply both to genetic diseases such as Wilson's, and to derangements caused by lifestyle such as the changes associated with obesity / lack of exercise.

Answer 13

Liver Disease/ Neuro: Untreated wilson disease may manifest as liver disease (toxic hepatitis) in childhood and neurologic problems(tremor, deteriation of school performance, psychiatric isssues) as young adults.

Answer 14

Wilson's disease: Wilson's disease or hepatolenticular degeneration is an autosomal recessive genetic disorder in which copper accumulates in tissues. The main sites of copper accumulation are the liver and the brain, and consequently liver disease and neuropsychiatric symptoms are the main features that lead to diagnosis.About half the people with wilson's disease have neurological or psychiatric symptoms.

Answer 15

Vague: It is very easy to overlook the subtle mental and movement changes that warn of Wilson's. The elevated liver enzymes, possible Fanconi syndrome revealed on urinalysis, and/or the low-grade hemolysis cause no symptoms. By the time the KF rings are visible in the eye, the disease is advanced. Have a high index of suspicion.

Answer 16

Genetic defect: Wilson disease is a defect in the bodies handling of copper ingested in the diet.A gene is inherited from both asymptomatic carrier parents that encodes the wrong information on copper metablolism.The proper carrier protein that is supposed to bind and take copper to be removed is not formed. Over time the copper begins to accumulate in tissues in toxic amounts.

Answer 17

Genes: Wilson's disease is due to mutations in the wilson disease protein (atp7b) gene. Wilson's - konovalov's disease or hepatolenticular degeneration is an autosomal recessive genetic disorder in which copper accumulates in tissues like the liver and cornea; this manifests as neurological or psychiatric symptoms and liver disease. It is treated with medication and sometimes liver transplant.

Answer 18

Genetic mutation: With defective ATP7B gene product, liver cells cannot get their copper into the bile, and generally the processing of copper is deranged.

how serious is wilson's disease?

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