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         Hepatolenticular Degeneration:     more detail
  1. Studies of protein metabolism in hepatolenticular degeneration (Research report) by Frank L Iber, 1956
  2. Diseases of the Nervous System described for Practitioners and Students with Chapters on the Neurological Complications of Liver Disease and Hepatolenticular Degeneration. by Francis Martin Rouse ; Walshe, John Michael Walshe, 1956-01-01
  3. Wilson's Disease: A Clinician's Guide to Recognition, Diagnosis, and Management by George J. Brewer, 2001-05-31
  4. Orphan Diseases and Orphan Drugs (Fulbright Papers, Proceedings of Colloquia, Vol 3) by I. Herbert Scheinberg, 1986-12
  5. Wilson's Disease by Tjaard Hoogenraad, 1996-07-15
  6. Wilson's Disease for the Patient and Family: A Patient's Guide to Wilson's Disease and Frequently Asked Questions about Copper by George J. Brewer M.D., 2002-04-09
  7. Liver-brain relationships by Ian A Brown, 1957
  8. Die Wilson'sche Krankheit (Westphal-Strümpell'sche Pseudosklerose): Eine Übersicht über die humangenetischen Forschungsergebnisse der Jahre 1967-1977 : Inaugural-Dissertation by Susanne Dworak, 1980
  9. Wilson's disease: Familial presentatin by Ying-tung Kung, 1983

1. Hepatolenticular Degeneration -- ECureMe.com
UPDATED SARS INFORMATION,Check health symptoms, make a self diagnosis, ask a doctor or veterinarian online, view an encyclopedia of diseases treatments,
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Hepatolenticular Degeneration
more about Hepatolenticular Degeneration

Wilson's disease
  • Wilson's disease is an inherited disease that causes problems within the liver, psychiatric problems, and occasionally leads to problems within the brain. It usually occurs in younger people. However, if detected early, it can be treated.

2. Dr. Rose's Peripheral BrainWILSON'S DISEASE (HEPATOLENTICULAR
WILSON'S DISEASE (hepatolenticular degeneration) I. Pathophysiology
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3. AllRefer Health - Wilson's Disease (Hepatolenticular Degeneration)
Wilson s Disease (hepatolenticular degeneration) information center covers causes, prevention, symptoms, diagnosis, treatment, incidence, risk factors,
http://health.allrefer.com/health/wilson-disease-info.html

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Alternate Names : Hepatolenticular Degeneration Definition Wilson's disease is an inherited disorder where there is excessive amounts of copper in the body. This causes a variety of effects, including liver disease and damage to the nervous system.
Central Nervous System
Copper Urine Test Wilson's disease is a rare inherited disorder. If both parents carry an abnormal gene for Wilson's disease, there is a 25% chance that each of their children will develop the disorder (i.e., it is an autosomal recessive disease). Wilson's disease causes the body to absorb and retain excessive amounts of copper. The copper deposits in the liver, brain, kidneys, and the eyes. The deposits of copper cause tissue damage, death of the tissues, and scarring, which causes the affected organs to stop functioning properly. Liver failure and damage to the central nervous system (brain, spinal cord) are the most predominant, and the most dangerous, effects of the disorder.

4. MedlinePlus Medical Encyclopedia Wilson's Disease
Central nervous system. Copper urine test Alternative names Return to top. hepatolenticular degeneration Definition Return to top
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5. AllRefer Health - Wilson's Disease Pictures & Images (Hepatolenticular Degenerat
Wilson s Disease (hepatolenticular degeneration) pictures and images. Alternate Names hepatolenticular degeneration
http://health.allrefer.com/health/wilson-disease-pictures-images.html

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Alternate Names : Hepatolenticular Degeneration Click on an image thumbnail to view full size
Central Nervous System The central nervous system is comprised of the brain and spinal cord. The peripheral nervous system includes all peripheral nerves.
Copper Urine Test The copper urine test is performed by collecting urine at specific times for a 24-hour period. The urine is tested for the amount of copper present. The copper urine test is used to determine the presence of Wilson disease, a sometimes fatal condition in which the buildup of excess copper damages the liver, and eventually the kidneys, eyes and brain.
Jump to another section Definition
Wilson's Disease Prevention
Wilson's Disease Treatment Wilson's Disease Prognosis ... Calling Your Health Care Provider Topics that might be of interest to you Anemia Cirrhosis Delirium Dementia ... WBC Count Other Topics Abdomen - Swollen Confusion Contracture Deformity Hand Tremor ... Main Page of Wilson's Disease From Our Sponsors:

6. The Wilson's Disease Association International
Learn about this disease, about the association, resources for consultation, diagnosis and treatment. Also copper content in various foods.
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7. Hepatolenticular Degeneration - Cerebral Pseudosclerosis - Information Page With
A rare autosomal recessive disease characterized by the deposition of copper in
http://www.hon.ch/HONselect/RareDiseases/EN/C06.552.413.html
InitBulle("navy","#F8F8F8","#000066",1); HONcode sites All Web sites HONselect News ... Images HONselect Search English French German Spanish Portuguese
the word the part of word in MeSH term in MeSH term and description Information on "Hepatolenticular Degeneration": Medical hierarchy and definition Research Articles Web resources Medical Images Medical News Medical Conferences Clinical Trials Hierarchy English French German Spanish Portuguese
Hepatolenticular Degeneration Definition: A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN LIVER CORNEA ; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS TREMOR ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years.
Synonym(s): Cerebral Pseudosclerosis / Neurohepatic Degeneration / Pseudosclerosis / Wilson Disease /
See Related: Brain Adenosine Triphosphate Liver Cirrhosis Cornea ... New search
Web resources for "Hepatolenticular Degeneration" English French Spanish Portuguese = Site with HON description - = Site with a robot description info: enter the site: (click below) domain of the site: HONcode - eMedicine - Wilson Disease : Article by Rahil Shah, MD

8. Healthopedia.com - Wilson's Disease (Hepatolenticular Degeneration)
Wilson's Disease. Alternate Names hepatolenticular degeneration
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9. HONselect - Hepatolenticular Degeneration
English, hepatolenticular degeneration, Cerebral Pseudosclerosis - Neurohepatic Degeneration - Pseudosclerosis - Wilson Disease
http://www.hon.ch/HONselect/RareDiseases/C06.552.413.html
List of rare diseases: English Deutsch
Language:
MeSH term:
Accepted terms:
English: Hepatolenticular Degeneration - Cerebral Pseudosclerosis
- Neurohepatic Degeneration
- Pseudosclerosis
- Wilson Disease
- Hepatic Form of Wilson Disease
- Hepato-Neurologic Wilson Disease
Français: Dégénérescence hépatolenticulaire - Dégénérescence lenticulaire progressive - Hépato-strié, syndrome Deutsch: Hepatolentikuläre Degeneration - Morbus Wilson - Pseudosklerose - Wilson-Krankheit - Neurohepatische Degeneration - Zerebrale Pseudosklerose - Progressive lentikuläre Degeneration Español: Degeneración Hepatolenticular - Enfermedad de Wilson - Degeneración Neurohepática - Pseudoesclerosis Cerebral - Seudoesclerosis Português: Degeneração Hepatolenticular - Degeneração Neurohepática - Doença de Wilson - Pseudoesclerose Cerebral - Pseudosclerose HONselect ressources Definition: Yes Articles: Yes Images: No News: No Conferences: No Clinical trials: No Web sites: English Yes Français Yes Deutsch No Español Yes Português Yes Home About us Site map ... Contact http://www.hon.ch/HONselect/RareDiseases/C06.552.413.html Last modified: Wed May 18 2005

10. HONselect - Hepatolenticular Degeneration
Language MeSH term Accepted terms English hepatolenticular degeneration
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11. Wilson's Disease (Hepatolenticular Degeneration)
Wilson s Disease (hepatolenticular degeneration) is a genetic disorder that is fatal unless detected and treated before serious illness develops from copper
http://www.geocities.com/nutriflip/Diseases/WilsonsDisease.html
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Wilson's Disease (Hepatolenticular Degeneration)
Wilson's Disease (Hepatolenticular Degeneration) is a genetic disorder that is fatal unless detected and treated before serious illness develops from copper poisoning. Wilson's Disease affects one in thirty thousand people world wide. The genetic defect causes excessive copper accumulation. Small amounts of copper are essential as vitamins. Copper is present in most foods, and most people get much more than they need. Healthy people excrete copper they don't need, but Wilson's Disease patients cannot. The gene for Wilson's disease (ATP7B) was mapped to chromosome 13. The sequence of the gene was found to be similar to sections of the gene defective in Menkes disease, another disease caused by defects in copper transport. The liver of a person who has Wilson's disease does not release copper into bile as it should. Bile is a liquid produced by the liver that helps with digestion. As the intestines absorb copper from food, the copper builds up in the liver and injures liver tissue. Eventually, the damage causes the liver to release the copper directly into the bloodstream, which carries the copper throughout the body. The copper buildup leads to damage in the kidneys, brain, and eyes. If not treated, Wilson's disease can cause severe brain damage, liver failure, and death. Symptoms usually appear between the ages of 6 and 20 years, but can begin as late as age 40. The most characteristic sign is the Kayser-Fleischer ringa rusty brown ring around the cornea of the eye that can be seen only through an eye exam. Other signs depend on whether the damage occurs in the liver, blood, central nervous system, urinary system, or musculoskeletal system. Many signs would be detected only by a doctor, like swelling of the liver and spleen; fluid buildup in the lining of the abdomen; anemia; low platelet and white blood cell count in the blood; high levels of amino acids, protein, uric acid, and carbohydrates in urine; and softening of the bones. Some symptoms are more obvious, like jaundice, which appears as yellowing of the eyes and skin; vomiting blood; speech and language problems; tremors in the arms and hands; and rigid muscles.

12. Wilson('s) Disease (Hepatolenticular Degeneration)
Wilson('s) Disease (hepatolenticular degeneration) Wilson's Disease Resources Information. Wilsons disease. Wilson disease. Wilson's Disease
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13. Hepatolenticular Degeneration
Notes for physicians on Wilson Disease (hepatolenticular degeneration) covering diagnosis, clinical description, differential diagnosis, management,
http://omni.ac.uk/browse/mesh/D006527.html
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Hepatolenticular Degeneration
other: Hemochromatosis GeneReviews : Wilson disease Notes for physicians on Wilson Disease (Hepatolenticular Degeneration) covering diagnosis, clinical description, differential diagnosis, management, molecular genetics and genetic counselling. Updated in April 2003, this resource forms part of GeneReviews (formerly GeneClinics profile), a peer-reviewed clinical genetic information resource that is funded by the US National Institutes of Health (NIH) and produced by the University of Washington, Seattle. This resource contains a summary and bibliographical references of the review. Hepatolenticular Degeneration / genetics
Last modified: 02 Sep 2005

14. ? Wilson Disease
Alternative Names hepatolenticular degeneration Causes, incidence, and risk factors
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15. Hepatolenticular Degeneration - Definition Of Hepatolenticular Degeneration By T
Definition of hepatolenticular degeneration in the Online Dictionary. Meaning of hepatolenticular degeneration. What does hepatolenticular degeneration
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hepatolenticular degeneration
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Cite / link Email Feedback Thesaurus Legend: Synonyms Related Words Antonyms Noun hepatolenticular degeneration - a rare inherited disorder of copper metabolism; copper accumulates in the liver and then in the red blood cells and brain Wilson's disease congenital disease genetic abnormality genetic defect ... inherited disorder - a disease or disorder that is inherited genetically Mentioned in References in classic literature No references found No references found Dictionary/thesaurus browser Full browser hepatocellular carcinoma Hepatocystic hepatocyte hepatoflavin ... hepatojugular reflux hepatolenticular degeneration Hepatology hepatoma hepatomegaly Hepatorenal ... hepatojugular reflux hepatolenticular degeneration hepatolith hepatolithectomy hepatolithiasis hepatology ... Hepatophyta Word (phrase): Word Starts with Ends with Definition Free Tools: For surfers: Browser extension Word of the Day NEW!

16. Wilsons Disease
Med.Sci 532 StructureFunction Wilson Disease (hepatolenticular degeneration)
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17. Hepatolenticular Degeneration Topic - Unified Search Environment
hepatolenticular degeneration Topic Tree hepatolenticular degeneration MSH/MH/D006527 DXP/SY/NOCODE LCH/PT/U002175 MTHICD9/ET/275.1
http://www.use.hcn.com.au/portals/shared/subject.`Hepatolenticular Degeneration`
Hepatolenticular Degeneration Topic Tree Definition:
A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. Clinical features include LIVER CIRRHOSIS; LIVER FAILURE; SPLENOMEGALY; TREMOR; bradykinesia; DYSARTHRIA; CHOREA; MUSCLE RIGIDITY; Kayser-Fleischer rings (pigmented corneal lesions); ATAXIA; and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years. (From Adams et al., Principles of Neurology, 6th ed pp969-71) Synonyms and Source Vocabularies:
Hepatolenticular Degeneration
Progressive Lenticular Degeneration
Pseudosclerosis
Wilson Disease
WESTPHAL-STRUEMPELL PSEUDOSCLEROSIS
Cerebral Pseudosclerosis
Westphal-Strumpell Syndrome
Hepatocerebral Degeneration
Neurohepatic Degeneration Kinnier-Wilson Disease Gowers' chorea Westphal Strumpell disease familial hepatitis Westphal pseudosclerosis Hepato-Neurologic Wilson Disease Brain Diseases

18. Penn State Faculty Research Expertise Database (FRED)
Degeneration, hepatolenticular degeneration, Neurohepatic. Degeneration, Progressive Lenticular Degenerations, Hepatocerebral
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19. Wilson's Disease Hepatolenticular Degeneration - Patient UK
Wilson s Disease is a genetic disorder in which copper builds up in the body, mainly in the liver and brain. Without treatment, the build up of copper
http://www.patient.co.uk/showdoc/23068935/
Wilson's Disease
Hepatolenticular Degeneration
Wilson's Disease is a genetic disorder in which copper builds up in the body, mainly in the liver and brain. Without treatment, the build up of copper causes serious illness, eventually causing death. Treatment is with medication to remove the excess copper and/or to prevent a further build up of copper.
What is Wilson's disease? Wilson's disease is an condition where too much copper builds up in the body. It is a rare inherited disorder that affects about 1 in 30,000 people. It is named after Dr Samual Wilson who first described the disorder in 1912. If you inherit the genetic fault in Wilson’s disease, your body is not able to get rid of copper. Copper is a 'trace metal' which is in many foods. You need tiny amounts of copper to remain healthy. Normally, the body gets rid of any excess copper. People with Wilson's disease cannot get rid of excess copper and so it builds up in the body, mainly in the liver, the brain, the cornea and kidneys. Too much copper in the liver cells (the hepatocytes) is harmful and leads to liver damage. Damage to brain tissue mainly occurs in an area called the lenticular nucleus. Hence, Wilson's disease is also called 'Hepatolenticular Degeneration'. If left untreated, the damage becomes severe and eventually fatal. What causes Wilson's disease?

20. AllRefer Health - Wilson's Disease (Hepatolenticular Degeneration)
Wilson's Disease (hepatolenticular degeneration) information center covers causes, prevention, symptoms, diagnosis, treatment, incidence, risk
http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

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