This is my wiki on Wilson's disease. Its a genetic disorder where your body can not get rid of excess copper. Wilson's disease first effects your liver, central nervous system, or both.
Description
Wilson's disease is a genetic disorder where the human body can not correctly dispose of excess copper. Humans need a small amount of copper in their system,which is obtained by food, to stay healthy. But to much copper is not good for your body and in fact can be deadly. Wilson's disease first attacks your central nervous system, liver or both. Buildup of copper in your liver may cause ongoing liver disease. Acute liver failure rarely occurs, and most patients with Wilson's disease will experience symptoms of chronic liver disease. Some systems of are swelling of your liver or spleen, jaundice, fluid buildup in the legs or abdomen, bruising easily, and fatigue. Copper buildup in the central nervous system may result in neurological symptoms including problems with speech ,swallowing, and coordination,tremors, muscle stiffness,and behavioral changes. Some other signs of Wilson's disease are anemia, low platelet or white blood cell count ,slower blood clotting, high levels of amino acids protein, uric acid, and carbohydrates in urine , and premature osteoporosis and arthritis.[1] Wilson's disease is caused by build up of copper in the body,which is normally filtered by the liver into bile. People with Wilson's disease do release some copper ,but not at the normal rate that healthy people release the copper, this is due to a mutilation of the ATP7B gene. When the copper storage limit is reached the excess copper is released into the bloodstream and is therefore taken to the other organs like your brain, kidneys, and eyes.[1] This is an image of an eye with a Kayser
Fleischer rings which is a sign of Wilson's disease and
is the gold ring around
the colored part of your eye.[3]One out of 30,000 people have Wilson's Disease. This means that 9,000 people in the United States and 200,000 people worldwide will have Wilson's disease. The people who get Wilson's disease have inherited the ATP7B gene one from each parent. The people with only one copy of the abnormal gene do not have the symptoms. Most people with the disease have no known family history of it, a persons chances of having Wilson's disease increase if one or both of the parents have the disease. the disease equally effects men and women and symptoms usually appear between ages five to thirty five, although new cases have been reported in people between the ages of two to seventy five..[1] Treatment
The treatment for Wilson's disease is lots of therapy to reduce the amount of copper in your system, monitor your copper intake,
To test for Wilson's disease doctors monitor
the amount of copper in your system through urine samples. [4]
and treat any liver or central nervous system damage.There are several drugs that people with Wilson's disease can use,d-penicillamine (Cuprimine) and trientine hydrochloride (Syprine) are both drugs that make your organs release copper and put it back into your bloodstream. In turn most of that copper is then sent out of the body in the form of urine. An unfortunate side effect of both drugs is that your neurological problems can worsen which would be caused by the central nervous system sucking up the newly release copper. Some people using the drug d-penicillamine will also have some side effects like rash, and fever, side effects are lower in the drug trientine hydrochloride. The treatment for Wilson's disease is lifelong.[1] Because both the previous drugs may have serious side effects many patients prefer to use Zinc salt. It blocks further absorption without any serious side effects. [2] Diagnosing Wilson's disease
Wilson's disease is diagnosed with physical exams and lab test.
In the physical exams your doctor will look for any signs of Wilson's disease visible to the naked eye. A special light is used to look for Kayser-Fleischer rings. Kayser-Fleischer rings can be seen in almost all patients who have signs of neurological damage. Only about half of the people however only showing signs of liver damage will have Kayser-Flischer rings.
The amount of copper in the blood, urine, and liver tissue are shown in lab tests. most of the people who have Wilson's disease will have a lower level of copper in their blood and a lower level of corresponding ceruloplasmin. Corresponding ceruloplasmin is a protein which carries copper in the bloodstream. you can test this. A 24-hour urine collection will show an increase in copper the urine in most patients who show symptoms of Wilson's disease. A liver biopsy can show if the liver is holding too much copper. In a liver biopsy a doctor will remove a small sample of liver and tests it for the disease by looking at it under a microscope, there is a particular pattern for Wilson's disease[1]
Research
The National Institute of Diabetes and Digestive and Kidney Diseases sponsors and dose research for Wilson's disease.The U.S. Food and Drug Administration is looking at a new anti-copper drug. It is called tetrathiomolybdate (Coprexa). A National Institutes of Health-supported clinical trial found that tetrathiomolybdate was just as effective as trientine hydrochloride when it comes to removing copper but it has less risk of making neurological symptoms get worse...[1]
There are over two hundred and seventy completed and active Wilson's disease research studies. [5] This is the seal for the national institute of health [9]
Current Events
Now scientists realize that carriers have a bigger risk of copper overload when exposed to chronically high levels in their diet. [6] To test this scientists like Katrina Allen of the Murdoch Children's Research Institute, at the University of Melbourne, Royal Children's Hospital, Parkville, Victoria, Australia, and colleagues there and at Deakin University, Burwood, Victoria, and RMIT University, in Bundoora, Victoria,test small aminals such as mice to see if this is true. [6]
The mice that carrie the gene of a disease with simmilar effects to Wilsons disease are known as toxic milk mice. [6]
Katrina Allen first mixed the mouse's water with 300 milligrams per litre of copper for 4-7, 8-11, 12-15, or 16-20 months.The mouse's liver, spleen, kidney, and brain tissue were analysed using atomic absorption spectroscopy at the end of each period to figure out copper concentration. The researchers also measured the total liver condition and enzyme activity in the plasma for ceruloplasmin oxidase. They found that this level of chronic copper in the diet of the toxic milk mice significantly increased copper in the liver in the toxic milk heterozygous (carriers) and toxic milk homozygous (with symtoms) mice. The mice that were not toxic milk mice were resistant to the toxic effects of the increased intake of copper. [6]
This is one of the tested mice [6]
Genetics
This is a picture of the mutated ATP7B gene [3]
As you know, to inherit Wilsons disease the gene has to be present in both parents, and passed to the effected child. The gene that is responsible for Wilsons disease is located on chromosome 13, the gene is called ATP7B. Wilsons disease occurs many times because of a spontanious mutation disease but can also simply be transmited thrughout the generations. [7]
There have been more than thirty different mutations identified thus far. Therefore, it has been difficult to come up with a specific genetic screening test for the Wilson’s disease. But if a specific mutation is recognized in a family, a genetic diegnosis is possible. This will possibly help with identifing symtom free relatives that are effected. This way they can get treated befor they become sick or handicapped. [7]
People who have only one of the required two
genes are called carriers or heterozygotes. They might have small but medicaly insignificant abnormities of copper metabolism. But they do not become sick or need medical atention. [7]
There is a firty percent chance
of this person having Wilson's
disease and a fifty percent
chance of this person being just
a carrier by Larkin Murray A.K.A me!
A Day In The Life
This is just a paragraph explaining what happend first
As a child you would be relatively normal fot the begining of your life. Although you would notice that you were gettind sick, because you would have symtoms like fevers, inflamed glands and vomiting attacks. You would also be brusing very easly and badly, mabey one day you would fall two feet off the jungle jim and be brused dor weeks. But life would go on, you would act very mormal and seem perfectly fine although you were becoming jandice, your parents would not notice because it was so gradual. It is around this time when you liver could possibly fail for the very first time. [8]
This person would have a difficult childhood and life. They would have to go through treatment diegnosis and mabey even a liver transplant. [8]
This is a removed
liver due to a transplant [8] But a average day in the life of a person with Wilsons disease would pretty much be the same as an averager day for a very healthy person. The only difference would be they would have to take medications and they might also go in for a check up every once in a while. Unless the terminated disease comes back they would live a very normal adult life (on the average days). [8]for video go to
Vocabulary
jaundice: yellowing of the skin of whites of the eyes
fatigue: tiredness
anemia: lack of power, vitality or colorfulness
platelet: small plate like body
bile: a liquid secreted by the liver
chronically: having a long deration
acute: sharp or severe
enzyme: any of numerous complex proteins that are produced by living cells and catalyze specific biochemical reactions at body temperatures
Slideshow
Bibliography
1. "Wilson's disease." digestive diseases home. NDDIC. 17 Jan 2009 <http://digestive.niddk.nih.gov/ddiseases/pubs/wilson/>.
2."NINDS Wilsons Disease information page." National Institute of nerological disorders and stroke. National institutes of health. 17 Jan 2009 <http://www.ninds.nih.gov/disorders/wilsons/wilsons.htm>. 3."Wilson's disease." Wikipedia. 14 Jan 2009. 16 Jan 2009 <http://en.wikipedia.org/wiki/Wilson%27s_disease>. 4."Wilson's Disease." Medline Plus. National Library of Medicine/National Institutes of Health. 17 Jan 2009 http://www.nlm.nih.gov/medlineplus/ency/article/000785.htm#visualContent. 5."Wilson's disease." clinicaltrials.gov. U.S. national instatute of health. 20 Jan 2009 http://www.clinicaltrials.gov/ct2/results?term=Wilson%27s+disease. 6. Bradley, David. "Copper blues and the toxic mouse trap ." Atomic Spectroscopy. 15 sept 2007. WILEY. 21 Jan 2009 <http://images.google.com/imgres?imgurl=http://www.spectroscopynow.com/coi/cda/download.cda%3Fid%3D17081&imgrefurl=http://www.spectroscopynow.com/coi/cda/landing.cda%3Ftype%3DFeature%26d-49489-p%3D2%26chId%3D1&usg=__ycCixTQ6p-HjzpDp2j8sHr7VSNs=&h=70&w=70&sz=2&hl=en&start=47&sig2=EjM88rEbZE2wF0BjBGyb2A&um=1&tbnid=e2pxt9Na1lsO3M:&tbnh=68&tbnw=68&ei=acx3SZP_I5LSMejV0CQ&prev=/images%3Fq%3Dwilson%2527s%2Bdisease%2Bresearchers%26start%3D40%26ndsp%3D20%26um%3D1%26hl%3Den%26rls%3Dcom.microsoft:en-us:IE-SearchBox%26rlz%3D1I7GFRC%26sa%3DN>. 7. "Genetics." The genetics and inheritance of Wilson's disease. Wilson's disease associstion. 23 Jan 2009 http://www.gatepharma.com/Galzin/genetics.pdf.
8.My life with wilsons disease. 23 Jan 2009 <http://images.google.com/imgres?imgurl=http://www.livers.org.nz/Our_Stories/Bethli/OffalBag.jpg&imgrefurl=http://www.livers.org.nz/Our_Stories/Bethli/Bethli.htm&usg=__rd5VUQroLPi-ii_yVL-NIiAo2cM=&h=331&w=441&sz=20&hl=en&start=132&sig2=qEPRBf0Wwkfnc0iNU8u2Nw&um=1&tbnid=sQQl1C7lCVayYM:&tbnh=95&tbnw=127&ei=sXp6SZ6JFtXkmQftxZUn&prev=/images%3Fq%3Dwilson%2527s%2Bdisease%26start%3D120%26ndsp%3D20%26um%3D1%26hl%3Den%26rls%3Dcom.microsoft:en-us:IE-SearchBox%26rlz%3D1I7GFRC%26sa%3DN>. References
1. "Wilson's disease." digestive diseases home. NDDIC. 17 Jan 2009 <http://digestive.niddk.nih.gov/ddiseases/pubs/wilson/>. 2."NINDS Wilsons Disease information page." National Institute of nerological disorders and stroke. National institutes of health. 17 Jan 2009 <http://www.ninds.nih.gov/disorders/wilsons/wilsons.htm>. 3."Wilson's disease." Wikipedia. 14 Jan 2009. 16 Jan 2009 <http://en.wikipedia.org/wiki/Wilson%27s_disease>. 4."Wilson's Disease." Medline Plus. National Library of Medicine/National Institutes of Health. 17 Jan 2009 http://www.nlm.nih.gov/medlineplus/ency/article/000785.htm#visualContent. 5."Wilson's disease." clinicaltrials.gov. U.S. national instatute of health. 20 Jan 2009 http://www.clinicaltrials.gov/ct2/results?term=Wilson%27s+disease. 6. Bradley, David. "Copper blues and the toxic mouse trap ." Atomic Spectroscopy. 15 sept 2007. WILEY. 21 Jan 2009 <http://images.google.com/imgres?imgurl=http://www.spectroscopynow.com/coi/cda/download.cda%3Fid%3D17081&imgrefurl=http://www.spectroscopynow.com/coi/cda/landing.cda%3Ftype%3DFeature%26d-49489-p%3D2%26chId%3D1&usg=__ycCixTQ6p-HjzpDp2j8sHr7VSNs=&h=70&w=70&sz=2&hl=en&start=47&sig2=EjM88rEbZE2wF0BjBGyb2A&um=1&tbnid=e2pxt9Na1lsO3M:&tbnh=68&tbnw=68&ei=acx3SZP_I5LSMejV0CQ&prev=/images%3Fq%3Dwilson%2527s%2Bdisease%2Bresearchers%26start%3D40%26ndsp%3D20%26um%3D1%26hl%3Den%26rls%3Dcom.microsoft:en-us:IE-SearchBox%26rlz%3D1I7GFRC%26sa%3DN>.
7. "Genetics." The genetics and inheritance of Wilson's disease. Wilson's disease associstion. 23 Jan 2009 http://www.gatepharma.com/Galzin/genetics.pdf. 9. "National Institutes of Health (NIH) Podcast." PodBean.com. 23 Jan 2009 <http://images.google.com/imgres?imgurl=http://www.podbean.com/image-logos/19918_logo.gif&imgrefurl=http://www.podbean.com/search%3Fk%3Dtag%26v%3Dmedical%26page%3D3&usg=__MHFgEwJdKOgV3Wqx8QYTBYgmWRg=&h=300&w=300&sz=8&hl=en&start=14&sig2=wh6scP0FHFwDPPzRDzs8_g&um=1&tbnid=Zt2-xhPgyNQCLM:&tbnh=116&tbnw=116&ei=kIR6Sd77C9KwmQe10ZE0&prev=/images%3Fq%3DA%2BNational%2BInstitutes%2Bof%2BHealth%26um%3D1%26hl%3Den%26rls%3Dcom.microsoft:en-us:IE-SearchBox%26rlz%3D1I7GFRC%26sa%3DG>. | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
the colored part of your eye.[3]





good job
keep up the good work
Great job who ever this is
Keep going
This is larkin right?
GREAT WORK! i cant wait to learn more!
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you have a lot of stuff in some of the sections! Keep it up!
this is really good keep going its gunna be great
nice larkin. scary picture, but the work is really good.
This is good Larken and don't call me billy!
its great, but u know where u put all of these: [1]? after 1 u need to do 2 and after 2 u need to put the three. is in number order. if u put all of the same number, ur readers cant find the correct site in the citation list.
i have all those 1s because all that info came from the site listed as 1. and i do also have a 2 a 3 a 4 and a 5
very good site. i hope u get A+
looks great, i really like the colors in ur punnet square
did your computer get fixed? hopefully, good work, way to pull through