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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
Hospital Directory. hepatolenticular degeneration. more about hepatolenticular degeneration. Wilson's disease
http://www.ecureme.com/emyhealth/data/Hepatolenticular_Degeneration.asp
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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.
    Symptoms depend on where the copper is deposited.

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. HONselect - Hepatolenticular Degeneration
    English hepatolenticular degeneration, Cerebral Pseudosclerosis - Neurohepatic Degeneration - 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: No Web sites: English Yes Français Yes Deutsch No Español Yes Português Yes Home About us Site map ... HONewsletter http://www.hon.ch/HONselect/RareDiseases/C06.552.413.html Last modified: Wed Apr 28 2004

    4. HONmedia - Medical Images (D)
    Heart Valve Diseases (1), hepatolenticular degeneration (1). hepatolenticular degeneration (1), Hernia, Diaphragmatic, Traumatic (1).
    http://www.hon.ch/HONmedia/D.html
    All Web sites HONcode sites HONselect News ... Z Search HONmedia:
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    French version

    List of images with terms beginning by " D DNA DNA Probes DNA Replication DNA Restriction Enzymes ... Dystocia Other images with terms beginning by " D AIDS D ementia Complex Addison's ... HONewsletter http://www.hon.ch/HONmedia/D.html Last modified: Wed Sep 24 2003

    5. ClinicalTrials.gov - Information On Clinical Trials And Human Research Studies:
    By Disease Heading Nervous System Diseases hepatolenticular degeneration
    http://clinicaltrials.gov/ct/screen/BrowseAny;jsessionid=8F3912DE71CEBAF29DC83D1

    6. Hepatolenticular Degeneration
    hepatolenticular degeneration. text version of the review requires brief registration. hepatolenticular degeneration / genetics;.
    http://omni.ac.uk/browse/mesh/C0019202L0019202.html
    low graphics
    Hepatolenticular Degeneration
    broader: Basal Ganglia Diseases Liver Diseases Movement Disorders other: Angelman Syndrome Canavan Disease Cockayne Syndrome Fatty Liver ... GeneReviews : Wilson disease Notes for physicians on Wilson Disease (Hepatolenticular Degeneration) covering diagnosis, clinical description, differential diagnosis, management, molecular genetics and genetic counselling. Reviewed during October 1999, 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, free access to the full-text version of the review requires brief registration. Hepatolenticular Degeneration / genetics
    Last modified: 27 May 2004

    7. ClinicalTrials.gov - Information On Clinical Trials And Human Research Studies:
    Diseases and Abnormalities at or before Birth hepatolenticular degeneration
    http://clinicaltrials.gov/ct/screen/BrowseAny?path=/browse/by-condition/hier/BC1

    8. NeuroGate.com
    Search results for "hepatolenticular degeneration" NO MATCHES FOUNDPlease select a different keyword or category Search AltaVista for 'hepatolenticular degeneration' Global Search
    http://www.neurogate.com/neuro/result.php3?search=Hepatolenticular Degeneration&

    9. MedWebPlus Subject Hepatolenticular Degeneration

    http://medwebplus.com/subject/Hepatolenticular_Degeneration
    Main About MWP Contribute to MWP Contact Us
    A service of Flexis, Inc. Welcome to MedWebPlus 2.3! A free service to help you find health sciences information quickly and easily.
    Hepatolenticular Degeneration
    Broader Terms:
    All Diseases
    Liver Diseases
    Related Terms:
    Alzheimer Disease
    Liver Neoplasms Phenylketonuria Hepatitis ... Tourette Syndrome
    Focussed Subsets:
    Enzymology
    Genetics therapy
    Web Sites:
    Entry GO Wilson's Disease Association GO WILSONS-LIST@LISTSERV.ACSU.BUFFALO.EDU Archives (Wilson's Disease Discussion Group)
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    10. Wilson's Disease - Learn More From MedlinePlus
    Wilson's Disease, hepatolenticular degeneration, hepatolenticular degeneration, Abnormalities, Digestive System
    http://rdre1.inktomi.com/click?u=http://www.nlm.nih.gov/medlineplus/wilsonsdisea

    11. MedWebPlus Subject Periodicals Hepatolenticular Degeneration

    http://medwebplus.com/subject/Periodicals/Hepatolenticular_Degeneration
    Main About MWP Contribute to MWP Contact Us
    A service of Flexis, Inc. Welcome to MedWebPlus 2.3! A free service to help you find health sciences information quickly and easily.
    Periodicals Hepatolenticular Degeneration
    Broader Terms:
    All Diseases
    Liver Diseases
    Related Terms:
    Alzheimer Disease
    Liver Neoplasms Phenylketonuria Hepatitis ... Tourette Syndrome
    Focussed Subsets:
    Enzymology
    Genetics therapy
    Web Sites:
    No web sites found.
    Flexis, Inc.
    Privacy statement
    Powered by y-Base

    12. AllRefer Health - Wilson's Disease (Hepatolenticular Degeneration)
    Wilson s Disease (hepatolenticular degeneration) information center covers causes, prevention, symptoms, diagnosis, treatment, incidence, risk factors, signs
    http://health.allrefer.com/health/wilson-disease-info.html
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    You are here : AllRefer.com Health Wilson's Disease
    Wilson's Disease
    Definition Prevention
    Treatment

    Expectations or Prognosis
    ...
    Go To Main Page
    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.

    13. AllRefer Health - Wilson's Disease (Hepatolenticular Degeneration)
    Wilson's Disease (hepatolenticular degeneration) information center covers causes, prevention, symptoms, diagnosis, treatment, incidence, risk factors, signs, tests, support groups, complications
    http://www.1uphealth.com/health/wilson_disease_info.html
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    You are here : AllRefer.com Health Wilson's Disease
    Wilson's Disease
    Definition Prevention
    Treatment

    Expectations or Prognosis
    ...
    Go To Main Page
    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.

    14. AllRefer Health - Wilson's Disease Support Groups (Hepatolenticular Degeneration
    Wilson s Disease (hepatolenticular degeneration) information center covers Support Groups. Alternate Names hepatolenticular degeneration.
    http://health.allrefer.com/health/wilson-disease-support.html
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    You are here : AllRefer.com Health Wilson's Disease : Support Groups for Wilson's Disease
    Wilson's Disease
    Definition Prevention
    Treatment

    Expectations or Prognosis
    ... Complications
    Support Groups Calling Your Health Care Provider
    Go To Main Page
    Alternate Names : Hepatolenticular Degeneration Wilson's Disease Support Groups Wilson disease support groups can be found at www.geneticalliance.org
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    Wilson's Disease Prevention

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    ... Wilson's Disease Complications Wilson's Disease Support Groups 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 ... Weakness Review Date : 6/3/2003 Reviewed By : A.D.A.M. editorial. Previous review by David G. Brooks, M.D., Ph.D., Division of Medical Genetics, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network (8/31/2001).

    15. AllRefer Health - Wilson's Disease (Hepatolenticular Degeneration) Information
    Wilson's Disease (hepatolenticular degeneration) information center covers definition, alternative names, Overview, Causes, Risk Factors, Symptoms Signs, Diagnosis Tests, Treatment, Support
    http://www.1uphealth.com/health/wilson_disease.html
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    Wilson's Disease
    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 Jump to a section
    Definition

    Prevention

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    Expectations or Prognosis
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    Delirium Dementia ... WBC Count Other Topics Abdomen - Swollen Confusion Contracture Deformity Hand Tremor ... Weakness From Our Sponsors: A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's

    16. 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.

    17. 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.
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    18. HEPATOLENTICULAR DEGENERATION - Meaning And Definition Of The Word
    Medical Dictionary. Search Dictionary hepatolenticular degeneration Dictionary Entry and Meaning. WordNet Dictionary. Definition n
    http://www.hyperdictionary.com/dictionary/hepatolenticular degeneration
    English Dictionary Computer Dictionary Thesaurus Dream Dictionary ... Medical Dictionary
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    HEPATOLENTICULAR DEGENERATION: Dictionary Entry and Meaning
    WordNet Dictionary Definition: [n] a rare inherited disorder ... brain Synonyms: Wilson's disease See Also: congenital disease genetic abnormality genetic defect genetic disease ... inherited disorder Biology Dictionary Definition: A recessive genetic disease in which affected individuals can't process and excrete copper properly; it results in liver and brain damage due to the metal's accumulation. Synonyms: Wilson's disease HOME ABOUT HYPERDICTIONARY

    19. DOE Document - THE USE OF $sup 64$Cu TO DIAGNOSE HEPATOLENTICULAR
    Abstract Not Provided THE USE OF $sup 64$Cu TO DIAGNOSE hepatolenticular degeneration. $ $ .
    http://rdre1.inktomi.com/click?u=http://www.osti.gov/energycitations/product.bib

    20. LookSmart - Directory - Wilson's Disease Or Hepatolenticular Degeneration
    Wilson s Disease or hepatolenticular degeneration Find FAQs, guides and personal stories about this rare genetic copper-storage disorder.
    http://search.looksmart.com/p/browse/us1/us317837/us317920/us53948/us330917/us27
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    Wilson's Disease or Hepatolenticular Degeneration - Find FAQs, guides and personal stories about this rare genetic copper-storage disorder.
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  • allRefer Health - Hepatocerebral Degeneration Information
    Chronicles facts on the brain disorder caused by liver damage, and reveals its tests, symptoms, causes, signs, prevention, expectations, complications, and treatment.
    allRefer Health - Wilson Disease Information

    Copper surpluses in the body characterizes hepatolenticular degeneration. Learn its signs, causes, symptoms, diagnosis, prevention, support groups, and treatment.
    Cincinnati Children's Hospital Medical Center - Wilson's Disease

    Provides facts regarding Wilson's Disease causes, symptoms, diagnosis, treatment, and survival rates.
    MedStudents - Metabolic Disorders, Wilson's Disease

    Fact sheet outlines the pathogenesis, symptoms, diagnosis, and management of the hereditary liver disorder.
    NIDDK - Wilson's Disease
    Courtesy of the Nat'l Institute of Diabetes and Digestive and Kidney Diseases, this overview provides additional resource links and addresses.
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