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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

21. Wilson's Disease And Hepatolenticular Degeneration Treatment At Mayo Clinic
Treatment for Wilson s disease and hepatolenticular degeneration to prevent acute liver failure at Mayo Clinic.
http://www.mayoclinic.org/wilsons-disease/
Home About Mayo Clinic Jobs Contact Us Mayo Clinic Locations: Arizona Florida Minnesota Mayo Clinic ... Medical Services Wilson's Disease Wilson's Disease Overview Diagnosis Treatment Options Appointments ... Medical Services
Treatment of Wilson's Disease at Mayo Clinic
(Synonyms: hepatolenticular degeneration) Mayo Clinic offers state-of-the-art care for patients with Wilson's disease, including pre-symptomatic screening, molecular testing and up-to-date drug and dietary treatments. The goal is to identify the disease and begin treatment before symptoms cause serious problems. Mayo Clinic Rochester has a special Wilson's Disease Clinic which brings together specialists from genetics, hepatology, neurology, psychiatry, laboratory medicine and nutrition to provide comprehensive care for patients and their families. The Wilson's Disease Clinic is a recognized Center of Excellence by the Wilson's Disease Association.
Diagnosis
Some estimate that at least half of Wilson's disease patients are never diagnosed and die of untreated disease. It is important to diagnose Wilson's disease early, before severe damage occurs. Symptoms of Wilson's disease typically begin to appear from early childhood through the 20s, with liver symptoms usually occurring first. Mayo Clinic laboratories offer comprehensive testing including urinary tests, blood tests, DNA testing and family screening. Read more about diagnosis of Wilson's disease
Treatment Options
Treatment for Wilson's disease is effective if diagnosis occurs before the onset of life-threatening symptoms. Typically a combination of oral medications and dietary changes is needed to halt disease progress. If treatment is stopped, the disease can be fatal. In the case of acute liver failure, a

22. Hepatolenticular Degeneration
hepatolenticular degeneration. Used for. cerebral pseudosclerosis. Used for. familial hepatitis Used for. hepatocerebral degeneration. Used for
http://crisp.cit.nih.gov/Thesaurus/00003721.htm
Prev Term: hepatoencephalomyelitis reovirus 3
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hepatolenticular degeneration
Used for:
cerebral pseudosclerosis
Used for:
familial hepatitis
Used for:
Gowers' chorea
Used for:
hepatocerebral degeneration
Used for:
Kinnier-Wilson disease
Used for:
neurohepatic degeneration
Used for:
Westphal pseudosclerosis
Synonym for:
Westphal Strumpell disease
Synonym for:
Wilson's disease
Broader Terms:
inborn metal metabolism disorder
Broader Terms:
liver disorder
Related Terms:
autosomal recessive trait
Scope Note:
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.
Term Number:
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23. Liver Information: Hepatolenticular Degeneration Caused By Excessive Deposition
USC Liver Transplant Surgery Program and Center for Liver Disease specializes in the surgical and medical management of the following disease entities
http://www.surgery.usc.edu/divisions/hep/livernewsletter-wilsondisease.html
Wilson Disease Wilson disease, also referred to as hepatolenticular degeneration, is caused by excessive deposition of copper within the liver, the central nervous system predominately in the basal ganglia and putamen, kidney, placenta and cornea. Patients can present with a wide range of clinical symptoms including movement disorders, speech impediments, psychiatric disorders, renal dysfunction and hemolytic anemia. In the liver, excess copper can cause chronic hepatitis with subsequent cirrhosis and eventual liver failure. Accelerated hepatic injury associated with acute Wilsonian crisis can lead to rapid liver failure and commonly requires liver transplantation. Like hemochromatosis, excessive accumulation of a divalent metal, copper, results in chemical generation of reactive oxygen intermediates causing cellular injury and organ damage. In summary, Wilson disease is an autosomal recessive disease caused by multiple mutations located throughout the WND gene. At this time, the diagnosis of Wilson disease is made by clinical and biochemical evidence of excess copper storage and not by genotyping studies. Haplotype analysis or screening for mutations identified in a Wilson disease patient can be used to identify siblings that may be carriers of the disease before occurrence of the clinical complication of excess copper storage.
Andrew Stolz, M.D.

24. Penn State Faculty Research Expertise Database (FRED)
, A rare autosomal recessive disease characterized Degeneration, Hepatolenticular, Degeneration, Neurohepatic......hepatolenticular degeneration.
http://fred.hmc.psu.edu/ds/retrieve/fred/meshdescriptor/D006527

25. Hepatolenticular Degeneration
Complete online version of The Encyclopaedia of Medical Imaging including text and images from The Encyclopaedia of Medical Imaging s eight book volumes
http://www.amershamhealth.com/medcyclopaedia/medical/Volume VII/HEPATOLENTICULAR

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26. Wilson's Disease
hepatolenticular degeneration; Lenticular Degeneration, Progressive. Disorder Subdivisions. None. General Discussion. Wilson’s disease is a rare genetic
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Who We Are About WebMD Site Map Health Topics Symptoms ... For a Complete Report Wilson's Disease Important It is possible that the main title of the report Wilson's Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms
  • Hepatolenticular Degeneration Lenticular Degeneration, Progressive
Disorder Subdivisions
  • None
General Discussion Wilson’s disease is a rare genetic disorder characterized by excess copper stored in various body tissues, particularly the liver, brain, and corneas of the eyes. The disease is progressive and, if left untreated, it may cause liver (hepatic) disease, central nervous system dysfunction, and death. Early diagnosis and treatment may prevent serious long-term disability and life threatening complications. Treatment is aimed at reducing the amount of copper that has accumulated in the body and maintaining normal copper levels thereafter. Resources WE MOVE (Worldwide Education and Awareness for Movement Disorders) 204 West 84th Street New York, NY 10024

27. HEPATOLENTICULAR DEGENERATION
hepatolenticular degeneration An inherited (autosomal recessive) disorder where there is excessive quantities of copper in the tissues, particularly the
http://www.medhelp.org/glossary2/new/GLS_2407.HTM
HEPATOLENTICULAR DEGENERATION - An inherited ( autosomal recessive ) disorder where there is excessive quantities of copper in the tissues, particularly the liver and central nervous system. Wilson's disease causes the body to absorb and retain copper. The copper deposits in the liver, brain, kidneys , and eyes. Complications include dementia and liver failure . Symptoms include jaundice vomiting , tremors, weakness, and slow stiff movements. Blood tests show serum ceruloplasmin is low. Medications are given to remove the excess copper from the body. Even with life-long treatment, disabling ( and life-threatening) side effects are common.
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28. Hepatolenticular Degeneration - General Practice Notebook
hepatolenticular degeneration. Wilson s disease is a rare, autosomal recessive disorder first described by KinnearWilson in 1912.
http://www.gpnotebook.co.uk/cache/1161101336.htm
hepatolenticular degeneration Wilson's disease is a rare, autosomal recessive disorder first described by Kinnear-Wilson in 1912. In the 1940's the defect in copper metabolism was noted. It is characterised by:
  • the accumulation of copper in the liver, basal ganglia, eye and other organs a low serum caeruloplasmin level
In Wilson's disease there are two fundamental defects in the metabolism of copper:
  • the rate of incorporation of copper into caeruloplasmin is reduced the rate of biliary excretion of copper is reduced
Classically, patients present with neurological disturbance, hepatic cirrhosis and Kayser-Fleischer rings. The condition is treatable and all young patients with cirrhosis should be screened.
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29. ► Wilson Disease
Wilson disease (hepatolenticular degeneration) is a rare inherited disorder, affecting approximately 1 out of 1 million people.
http://www.umm.edu/ency/article/000785.htm
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Wilson disease
Overview Symptoms Treatment Prevention Definition: An inherited disorder where there is excessive amounts of copper in the body, which causes a variety of effects including liver disease and damage to the nervous system.
Alternative Names: Hepatolenticular degeneration
Causes, incidence, and risk factors: Wilson disease (hepatolenticular degeneration) is a rare inherited disorder, affecting approximately 1 out of 1 million people. If both parents carry an abnormal gene for Wilson disease there is a 25% chance that each of their children will develop the disorder (i.e., Wilson is an autosomal recessive disease). It is most common in eastern Europeans, Sicilians, and southern Italians, but may occur in any group. The disorder most commonly appears in people under 40 years old. In children, the symptoms begin to be expressed by around 4 years old.
Wilson 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

30. Wilson's Disease
Synonyms. hepatolenticular degeneration; Lenticular Degeneration, Progressive. Disorder Subdivisions. None. General Discussion
http://www.bchealthguide.org/kbase/nord/nord26.htm
var hwPrint=1;var hwDocHWID="nord26";var hwDocTitle="Wilson's Disease";var hwRank="1";var hwSectionHWID="nord26-Header";var hwSource="en-caQ2_05";var hwDocType="Nord";
National Organization for Rare Disorders, Inc.
Wilson's Disease
Important
It is possible that the main title of the report Wilson's Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms
  • Hepatolenticular Degeneration Lenticular Degeneration, Progressive
Disorder Subdivisions
  • None
General Discussion
Resources
WE MOVE (Worldwide Education and Awareness for Movement Disorders)
204 West 84th Street
New York, NY 10024
USA
Tel: 2128758312
Fax: 2128758389
Tel: 8004376682
Email: wemove@wemove.org
Internet: http://www.wemove.org
Wilson's Disease Association International 1802 Brookside Drive Wooster, OH 44691 USA Tel: 3302641450 Fax: 5097576418 Tel: 8003990266 TDD: 5407431415 Email: wilsonsdiseaseassoc@yahoo.com Internet: http://www.wilsonsdisease.org March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue White Plains, NY 10605

31. Dense Kayser-Fleischer Ring In Asymptomatic Wilson's Disease (hepatolenticular D
Finelli PF, KayserFleischer ring hepatolenticular degeneration (Wilson s disease). Neurology 1995;451261–2.Medline; Rodman R, Burnstine M, Esmaeli B,
http://bjo.bmjjournals.com/cgi/content/full/86/1/114

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Vol Page [Advanced] This Article Extract Full Text (PDF) Submit a response ... Alert me if a correction is posted Services Email this link to a friend Similar articles in this journal Alert me to new issues of the journal Download to citation manager PubMed Articles by Sullivan, C. A. Articles by Shun-Shin, G A Related Collections Other ophthalmology British Journal of Ophthalmology
British Journal of Ophthalmology
LETTER
Dense Kayser-Fleischer ring in asymptomatic Wilson's disease (hepatolenticular degeneration)
Charlotte Anne Sullivan A Chopdar and G A Shun-Shin Department of Ophthalmology, Birmingham and Midlands Eye Hospital, City Hospital, Western Road, Birmingham B18 7QU, UK
Department of Ophthalmology, East Surrey Hospital, Surrey, UK
Department of Ophthalmology, Wolverhampton Eye Infirmary, Wolverhampton, West Midlands, UK Correspondence to:
Miss C A Sullivan, Birmingham and Midlands Eye Hospital, City Hospital, Western Road, Birmingham B18 7QU, UK

32. Dense Kayser-Fleischer Ring In Asymptomatic Wilson's Disease (hepatolenticular D
Keywords KayserFleischer ring; Wilson s disease; hepatolenticular degeneration. The Kayser-Fleischer ring is the single most important diagnostic sign in
http://bjo.bmjjournals.com/cgi/content/extract/86/1/114

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Vol Page [Advanced] This Article Full Text Full Text (PDF) Submit a response ... Alert me if a correction is posted Services Email this link to a friend Similar articles in this journal Alert me to new issues of the journal Download to citation manager PubMed Articles by Sullivan, C. A. Articles by Shun-Shin, G A Related Collections Other ophthalmology British Journal of Ophthalmology
LETTER
Dense Kayser-Fleischer ring in asymptomatic Wilson's disease (hepatolenticular degeneration)
Charlotte Anne Sullivan A Chopdar and G A Shun-Shin Department of Ophthalmology, Birmingham and Midlands Eye Hospital, City Hospital, Western Road, Birmingham B18 7QU, UK
Department of Ophthalmology, East Surrey Hospital, Surrey, UK
Department of Ophthalmology, Wolverhampton Eye Infirmary, Wolverhampton, West Midlands, UK Correspondence to:
Miss C A Sullivan, Birmingham and Midlands Eye Hospital, City Hospital, Western Road, Birmingham B18 7QU, UK

33. HighWire -- Browse Journals - Hepatolenticular Degeneration
Browse Journals publishing on hepatolenticular degeneration Journals focusing on hepatolenticular degeneration (in order by highest focus)
http://highwire.stanford.edu:4141/lists/topic_dir/608683/608684/615888/615939/61
THIS PREVIEW SITE IS FOR DEMONSTRATION PURPOSES ONLY details About Us Contact Us Help ... Sign in for more free features or create a free account Keywords: any all phrase more options... Authors: e.g. Smith, JS; Jones, D Citation: Year Vol Page Articles: HighWire-hosted only From My Favorite Journals only sign in All (including PubMed) Browse Journals publishing on Hepatolenticular Degeneration: (return to Topic List page) Hepatolenticular Degeneration
Sort by: Alphabet Frequency of articles in Hepatolenticular Degeneration Focus of journal on Hepatolenticular Degeneration What's this? Journals focusing on Hepatolenticular Degeneration (in order by highest focus) Journal of Medical Genetics info free ISSUES Postgraduate Medical Journal ... help
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34. Hepatolenticular Degeneration (syn. Of Wilson S Disease)
ThirdAid.com the world s first dedicated online patient to patient medical help community. i Wilson s disease /i is an autosomal recessive hereditary
http://www.thirdaid.com/conditions/hepatolenticular_degeneration.htm

35. Wilson's Disease (Hepatolenticular Degeneration) » Clinical And Experimental Op
Subscriptions Back Issues Contact Details. Journal Search. All, Author, Title. Journal of. Wilson s disease (hepatolenticular degeneration)
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36. Wilson’s Disease, Copper Overload, Hepatolenticular Degeneration
Wilson’s disease is a genetic disorder that results in excessive accumulation of copper in many parts of the body, particularly the liver.
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Welcome to the Truestar Health Encyclopedia Welcome to the Truestar Health Encyclopedia –the most comprehensive information database available on health, wellness, food, nutrition, vitamins and supplements. Use of our encyclopedia will enable you to make well-informed, responsible decisions for the promotion of your own health and wellness. Enter search items Also indexed as: Copper Overload, Hepatolenticular Degeneration copper Rating Nutritional Supplements Herbs Zinc Reliable and relatively consistent scientific data showing a substantial health benefit.
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37. Hepatolenticular Degeneration - Hepatolenticular Degeneration Products
hepatolenticular degeneration products offered at HerbalStand.com. View Cart hepatolenticular degeneration. What is hepatolenticular degeneration ?
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Hepatolenticular Degeneration
What is Hepatolenticular Degeneration ? A hereditary degenerative disease in which a deficiency of ceruloplasmin causes copper to accumulate in the liver, brain, and other tissues. Causes of Hepatolenticular Degeneration Liver damage can lead to the accumulation of ammonia and other toxic materials when the organ doesn't function properly to metabolize and excrete them.
Hepatocerebral degeneration and the neurological syndrome it causes may occur in any case of acquired liver failure including alcoholic hepatitis, autoimmune hepatitis, viral hepatitis, and others. Symptoms of Hepatolenticular Deneneration Symptoms may include:
  • tremor (shaking of arms, head)
  • ataxia (unsteady walking) difficulty walking chorea (uncontrolled body movements) myoclonus (spasm of muscles) asterixis impairment of intellectual function coma jaundice rigidity

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38. Healthopedia.com - Wilson's Disease (Hepatolenticular Degeneration)
Wilson s Disease. Alternate Names hepatolenticular degeneration. Overview, Causes, Risk Factors Symptoms Signs Diagnosis Tests Prevention
http://www.healthopedia.com/wilsons-disease/
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Wilson's Disease
Alternate Names : Hepatolenticular Degeneration Attribution Wilson's disease is an inherited inborn error of metabolism in which the body cannot process copper. What is going on in the body? Wilson's disease is caused by a defect in an enzyme, or special protein. This enzyme normally helps the body get rid of extra copper. The amount of copper in a normal diet is more than the body needs. In people with Wilson's disease, the extra copper cannot be excreted from the body. The copper can damage the liver, brain, kidney, cornea, and other organs. What are the causes and risks of the disease? Wilson's disease is an inherited disorder. The children of parents who carry the gene for the disorder are at risk. The liver disease usually starts between ages 8 and 12, while the brain symptoms occur later in life.

39. Healthopedia.com - Wilson's Disease: Prevention & Expectations (Hepatolenticular
Wilson s Disease. Alternate Names hepatolenticular degeneration. Wilson s Disease Symptoms Signs Diagnosis Tests Prevention Expectations
http://www.healthopedia.com/wilsons-disease/prevention.html
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You are here : Healthopedia.com Medical Encyclopedia Diseases and Conditions Wilson's Disease
Category : Health Centers Brain and Nervous System
Wilson's Disease
Alternate Names : Hepatolenticular Degeneration Wilson's Disease Attribution What can be done to prevent the disease? Nothing can be done to prevent Wilson's disease in someone who has already been born. Genetic testing can determine whether someone has the gene for Wilson's disease. Genetic counseling is useful for people with a family history of the disease. What are the long-term effects of the disease? If untreated, Wilson's disease causes death. A delay in treatment can cause permanent brain damage, hepatitis , or cirrhosis . If treatment is started early and continued for life, there may be no long-term effects. What are the risks to others?

40. PsychiatryOnline - Search Results
Search Results hepatolenticular degeneration. Book Results (0), Journal Results (6) Do a fulltext search for hepatolenticular degeneration
http://www.psychiatryonline.com/searchResult.aspx?rootterm=hepatolenticular dege

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