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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. HONselect - Hepatolenticular Degeneration
English hepatolenticular degeneration, Cerebral Pseudosclerosis - NeurohepaticDegeneration - Pseudosclerosis - Wilson Disease - Hepatic Form of Wilson
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: DEGENERESCENCE HEPATOLENTICULAIRE - DEGENERESCENCE LENTICULAIRE PROGRESSIVE - HEPATO-STRIE, SYNDROME Deutsch: Hepatolentikuläre Degeneration - Neurohepatische Degeneration - Pseudosklerose - Wilson-Krankheit - Zerebrale Pseudosklerose - Morbus Wilson - Progressive lentikuläre Degeneration Español: DEGENERACION HEPATOLENTICULAR - PSEUDOESCLEROSIS CEREBRAL - DEGENERACION NEUROHEPATICA - SEUDOESCLEROSIS - ENFERMEDAD DE WILSON Português: DEGENERACAO HEPATOLENTICULAR - PSEUDOESCLEROSE CEREBRAL - DEGENERACAO NEUROHEPATICA - PSEUDOSCLEROSE - DOENCA DE WILSON HONselect ressources Definition: Yes Articles: Yes Images: No News: No Conferences: No Clinical trials: Yes Web sites: English Yes Français No Deutsch No Español No Português Yes Home About us Site map ... HONewsletter http://www.hon.ch/HONselect/RareDiseases/C06.552.413.html Last modified: Thu Jul 25 2002

2. Dr. Rose's Peripheral Brain--WILSON'S DISEASE (HEPATOLENTICULAR DEGENERATION)
WILSON'S DISEASE (hepatolenticular degeneration). I. Pathophysiology
http://faculty.washington.edu/momus/PB/wilsonsd.htm
WILSON'S DISEASE (HEPATOLENTICULAR DEGENERATION)
I. Pathophysiology
  • An autosomal recessive defect in a copper-transporting ATP-ase that leads to impaired biliary excretion of copper The result is copper accumulation in the liver, and in later stages, the brain, cornea, and kidneys as well
  • II. Clinical presentation
  • Accumulation begins in infancy but usually presents clinically during adolescence Hepatic manifestations Most often presents with cirrhosis and portal hypertension 10-30% will have chronic active hepatitis Rarely, get fulminant hepatic failure Often get a pattern of recurrent mild hepatitis In some cases, liver injury is subclinical and dysfunction of other organs becomes apparent earlier Neuropsychiatric sx-motor sx (tremor, chorea, decreased movement), loss of coordination of fine movements; personality changes; sz are infrequent; sensory abnormalities don't seem to occur Hemolytic anemia (occurs when copper is released from liver into circulation in large amounts; usually accompanied by overt liver disease)-brief, self-limited
  • 3. Hepatolenticular Degeneration
    MeSH2001 Thesaurus Advanced Search Registration Help Disclaimer. hepatolenticular degeneration. Wilson's disease
    http://www.brisbio.ac.uk/ROADS/subject-listing/hepatolenticulardegeneration.html
    A collection of medical, dental and veterinary images for use in teaching. Home About the Archive FAQ Terms and Conditions ... Help
    Hepatolenticular Degeneration
    Wilson's disease Wilson's disease Wilson's disease Wilson's disease Wilson's disease Wilson's disease Wilson's disease Wilson's disease

    4. The Effects Of B.A.L. In Hepatolenticular Degeneration
    The effects of B.A.L. in hepatolenticular degeneration Pages 10 22 Part of the OUP Brain WWW service Click here to register with OUP. Last updated 13 May 97
    http://www3.oup.co.uk/jnls/supplements/braini/hdb/Volume_74/Issue_01/740010.sgm.
    Volume 74: January - December 1951
    Issue 1: March 1951
    Abstract
  • The effects of B.A.L. in hepatolenticular degeneration
  • JN Cumings Pages: Part of the OUP Brain WWW service
    General Information
    Click here to register with OUP. This page is maintained by OUP admin Last updated 13 May 97 Part of the OUP Journals World Wide Web service Oxford University Press, 1997

    5. HONselect - Liver Diseases
    Hepatic Vein Thrombosis, Hepatic VenoOcclusive Disease. hepatolenticular degeneration,Hepatopulmonary Syndrome. Hepatorenal Syndrome, Peliosis Hepatis.
    http://www.hon.ch/HONselect/Selection/C06.552.html
    HONselect: Introduction Search Browse Favourites List of Rare Disease add HONselect Contents on "Liver Diseases": MeSH hierarchy and definition Research Articles Web resources Medical Images Medical News Medical Conferences Clinical Trials Wondering about the holiday blues! MeSH Hierarchy English French German Spanish Portuguese MeSH Broader term(s) Diseases
    Digestive System Diseases
    Liver Diseases MeSH Narrow term(s) Cholestasis, Intrahepatic Fatty Liver Hepatitis Hypertension, Portal Liver Abscess Liver Cirrhosis Liver Diseases, Alcoholic Liver Diseases, Parasitic Liver Failure Liver Neoplasms Porphyria, Hepatic Focal Nodular Hyperplasia Hepatic Vein Thrombosis Hepatic Veno-Occlusive Disease Hepatolenticular Degeneration Hepatopulmonary Syndrome Hepatorenal Syndrome Peliosis Hepatis Porphyria, Erythrohepatic Tuberculosis, Hepatic Zellweger Syndrome Accepted term(s)
    -Acute Yellow Atrophy
    -Liver Dysfunction
    -Acute Yellow Atrophies
    -Atrophies, Acute Yellow
    Browse
    New search MEDLINE 's articles for " Liver Diseases English French German Spanish Portuguese All categories Selection by study category Refinement by recall or precision All recent articles
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    Etiology Prognosis Therapy by recall Diagnosis by recall Etiology by recall Prognosis by recall Therapy by precision Diagnosis by precision Etiology by precision Prognosis by precision Browse New search Web resources for " Liver Diseases English French info: enter the site: (click below) domain of the site: HepNet - Chronic Viral Hepatitis in the United States www.hepnet.com

    6. MedWebPlus Subject Hepatolenticular Degeneration
    Welcome to MedWebPlus 2.3! A free service to help you find health sciences informationquickly and easily. hepatolenticular degeneration Broader Terms
    http://www.medwebplus.com/subject/Hepatolenticular_Degeneration

    7. GeneReviews: Wilson Disease
    Medical Encyclopdia article about hepatolenticular degeneration A resource with information on over 4000 medical topics including hepatolenticular degeneration
    http://www.geneclinics.org/profiles/wilson
    Your browser does not support HTML frames so you must view Wilson Disease in a slightly less readable form. Please follow this link to do so.

    8. Wilson's Disease (Hepatolenticular Degeneration)
    Wilson's Disease (hepatolenticular degeneration). Wilson's Disease(hepatolenticular degeneration) is a genetic disorder that is
    http://www.diet-and-health.net/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.

    9. HEPATOLENTICULAR DEGENERATION
    hepatolenticular degeneration An inherited (autosomal recessive) disorder wherethere 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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    10. Wilson Disease (hepatolenticular Degeneration)
    Wilson disease (hepatolenticular degeneration) This disease is described in an article in GeneReviews.
    http://www.simulconsult.com/resources/c0519099.html
    Wilson disease (hepatolenticular degeneration)
    This disease is described in an article in GeneReviews
    SimulConsult
    Inc.

    11. Degeneration Articles, Support Groups, And Resources
    Y Z. Degeneration. Medical Glossary hepatolenticular degeneration;MACULAR DEGENERATION; SPINOCEREBELLAR DEGENERATION. Medical Library
    http://www.medhelp.org/HealthTopics/Degeneration.html
    [Health Topics A-Z]
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    B C D ... Z
    Degeneration

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    [Library Search] [Medical Forums] ... [Patient Network] Revised: 3/30/2003

    12. HEPATOLENTICULAR DEGENERATION
    hepatolenticular degeneration An inherited (autosomal recessive) disorder where there is excessive quantities of
    http://www.medhelp.org/glossary/new/gls_2213.htm
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    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.
    Med Help International
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    13. Liver Information: Hepatolenticular Degeneration Caused By Excessive Deposition
    Wilson disease, also referred to as hepatolenticular degeneration, is caused byexcessive deposition of copper within the liver, the central nervous system
    http://www.livertransplant.org/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.

    14. Hepatolenticular Degeneration
    Other characters. hepatolenticular degeneration . see Wilsons disease
    http://www.amersham-health.com/medcyclopaedia/Volume%20III%201/HEPATOLENTICULAR%
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    *For Medical Professionals only, registration required Hepatolenticular degeneration, see Wilsons disease
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    15. Wilson('s) Disease (Hepatolenticular Degeneration)
    HOME Wilson('s) Disease (hepatolenticular degeneration). Wilson's Disease Resources Information; wilsons; Wilsons disease; Wilson disease; Wilson's Disease;
    http://www.bdid.com/wilson.htm

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    16. Hepatolenticular Degeneration
    For Medical Professionals only. hepatolenticular degeneration . see Wilsons disease
    http://www.amersham-health.com/medcyclopaedia/Volume%20VII/HEPATOLENTICULAR%20DE
    Medcyclopaedia About Medcyclopaedia Amersham Health Search for: Type a word or a phrase.
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    *For Medical Professionals only, registration required Hepatolenticular degeneration, see Wilsons disease
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    17. Birth Disorder Information Directory - W
    Wilson('s) Disease (hepatolenticular degeneration) List of Sites. Wilson Turner(XLinked Mental Retardation) Syndrome (Mental Retardation, X-Linked, Syndromic 6
    http://www.bdid.com/defectw.htm

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    W Syndrome Waaler Aarskog Syndrome (Hydrocephalus with Costovertebral Dysplasia and Sprengel Anomaly) Waardenburg Syndrome

    18. E W A T E R . C O M - Minerals : Essential Angstrom Protocols : Hepatolenticular
    looking to contact us or a member of our staff? click here! YOU ARE HEREhome . hepatolenticular degeneration (Wilson's Disease). DESCRIPTION.
    http://www.ewater.com/health/minerals/protocols/wilsons.htm
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    DESCRIPTION
    A hereditary degenerative disease in which a deficiency of ceruloplasmin causes copper to accumulate in the liver, brain, and other tissues. It is characterized by cirrhosis, degeneration of the basal ganglia of the brain, and the disposition of green pigment in the periphery of the cornea.
    FORMULA
    Name Amt/Day Instructions Supply/Mo. Copper 1 tsp orally Germanium 2 TBSP orally Manganese 2 TBSP orally Sulfur 2 TBSP orally Water of Life (AM78) 1 TBSP orally, in the morning

    19. E W A T E R . C O M - Minerals : Essential Angstrom Protocols : Index
    Syndrome Heart Arrhythmia Heart Dysfunction Heart Failure Heavy Metal DetoxificationHepatitis C hepatolenticular degeneration (Wilsons Disease) Herpes Simplex
    http://www.ewater.com/health/minerals/protocols/
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    20. Hepatolenticular Degeneration -- ECureMe.com
    hepatolenticular degeneration, more about hepatolenticular degeneration,Wilson's disease. more about hepatolenticular degeneration, ..
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    Hepatolenticular Degeneration
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    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.
      Symptoms depend on where the copper is deposited.

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