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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. Hepatolenticular Degeneration
only. hepatolenticular degeneration,, Print this article, see Wilsonsdisease DR The Encyclopaedia of Medical Imaging Volume III1,
http://www.amershamhealth.com/medcyclopaedia/Volume III 1/HEPATOLENTICULAR DEGEN
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22. Hepatolenticular Degeneration
only. hepatolenticular degeneration,, Print this article, see Wilsonsdisease HP The Encyclopaedia of Medical Imaging Volume VII, Disclaimer
http://www.amershamhealth.com/medcyclopaedia/Volume VII/HEPATOLENTICULAR DEGENER
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*For Medical Professionals only, registration required Hepatolenticular degeneration, see Wilsons disease
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23. Wilson Disease (hepatolenticular Degeneration)
Wilson disease (hepatolenticular degeneration). References. Search Medlinefor hepatolenticular degeneration. Related differential diagnosis.
http://www.gentili.net/list4.asp?ID=118

24. Hepatolenticular Degeneration - General Practice Notebook
hepatolenticular degeneration. Wilson's disease is a rare, autosomalrecessive 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.
Click here for more information...

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acral lentiginous melanoma / normoblasts / uterus bicornis unicollis / wide pulsepressure / lepromatous leprosy / hepatolenticular degeneration / TAR syndrome
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26. 1Up Health > Wilson Disease > Prevention Of Wilson Disease (Hepatolenticular Deg
Comprehesive information on Wilson disease (hepatolenticular degeneration). Alternativenames hepatolenticular degeneration. Definition
http://www.1uphealth.com/health/wilson_disease_prevention.html
1Up Health Wilson disease Alternative Medicine Clinical Trials ... Health Topics A-Z Search 1Up Health Wilson disease Information Wilson disease Prevention Alternative names : Hepatolenticular degeneration 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.
Prevention
Genetic counseling is recommended for persons with a family history of Wilson's disease.
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27. 1Up Health > Wilson Disease (Hepatolenticular Degeneration) Information
Comprehesive information on Wilson disease (hepatolenticular degeneration).Covers info such as , alternative names, causes, incidence
http://www.1uphealth.com/health/wilson_disease.html
1Up Health Alternative Medicine Clinical Trials Health News ... Health Topics A-Z Search 1Up Health Wilson disease Information Guide Alternative names : Hepatolenticular degeneration 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.
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Causes, Incidence, and Risk Factors

Symptoms

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28. Alcoholic Hepatitis
Wilson's Disease hepatolenticular degeneration. Book, Home Page.
http://www.fpnotebook.com/GI61.htm
Home About Links Index ... Editor's Choice Paid Advertisement (click above). Please see the privacy statement Gastroenterology Hepatitis Infectious Disease ... Hepatitis E Assorted Pages Alcoholic Hepatitis Autoimmune Hepatitis Fulminant Hepatitis Nonalcoholic Fatty Liver ... Prevention of Liver Disease Progression Alcoholic Hepatitis Alcoholic Liver Disease Book Home Page Cardiovascular Medicine Dental Dermatology Emergency Medicine Endocrinology Gastroenterology General Medicine Geriatric Medicine Gynecology Hematology and Oncology HIV Infectious Disease Jokes Laboratory Neonatology Nephrology Neurology Obstetrics Ophthalmology Orthopedics Otolaryngology Pediatrics Pharmacology Prevention Psychiatry Pulmonology Radiology Rheumatology Sports Medicine Surgery Urology Chapter Gastroenterology Index Bowel Cirrhosis Constipation Dermatology Diarrhea Esophagus Examination Gynecology Hematology and Oncology Hepatitis Inflammatory Bowel Disease Infectious Disease Laboratory General Malabsorption Neurology Neonatology Obstetrics Pancreas Pediatrics Pharmacology Prevention Procedure Psychiatry Peptic Ulcer Disease Radiology Rectum Rheumatology Stomach Surgery Symptom Evaluation Page Hepatitis Index Alcohol Autoimmune Fulminant ID Viral ID Viral Type A ID Viral Type B ID Viral Type B Chronic Carrier ID Viral Type B Chronic Infection ID Viral Type C ID Viral Type D ID Viral Type E Steatosis Toxin
  • Pathophysiology Precursor to Cirrhosis Characteristics Hepatocyte Inflammation, degeneration, and necrosis
  • 29. Wilson Disease
    hepatolenticular degeneration. Wilson disease (hepatolenticular degeneration) isa rare inherited disorder, affecting approximately 1 out of 1 million people.
    http://www.pennhealth.com/ency/article/000785.htm
    Disease Injury Nutrition Poison ... Prevention
    Wilson disease
    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, necrosis (death of the tissues), and scarring, which causes decreased functioning of the organs affected. Liver failure and damage to the

    30. Wilson's Disease
    DISEASE (hepatolenticular degeneration; Lenticular Degeneration, Progressive;progressive lenticular degeneration; WestphalStruempell pseudosclerosis).
    http://www.icongrouponline.com/health/Wilson's_Disease.html
    ICON Health Publications
    Official Health Sourcebooks The Official Patient's Sourcebook
    on
    WILSON'S DISEASE

    (Hepatolenticular Degeneration; Lenticular Degeneration, Progressive; progressive lenticular degeneration; Westphal-Struempell pseudosclerosis) Revised and Updated for the Internet Age Paperback Book Order by phone: 800-843-2665 (within USA) 1-201-272-3651 (from outside USA) Electronic File * E-Book version sent via e-mail in 2 business days Electronic File * E-Book version sent via e-mail in 2 business days Pages Price $28.95(USD) ISBN Published Synopsis A comprehensive manual for anyone interested in self-directed research on Wilson's disease. Fully referenced with ample Internet listings and glossary. Related Conditions/Synonyms Hepatolenticular Degeneration; Lenticular Degeneration, Progressive; progressive lenticular degeneration; Westphal-Struempell pseudosclerosis Description Table of Contents Introduction Overview Organization Scope Moving Forward PART I: THE ESSENTIALS Chapter 1. The Essentials on Wilson’s Disease: Guidelines Overview What Is Wilson’s Disease?

    31. ACP-ASIM Online - Medicine In Quotations Online
    Subject hepatolenticular degeneration (Wilson disease). Contributor Huth,Edward J. Subject hepatolenticular degeneration (Wilson disease).
    http://www.acponline.org/cgi-bin/medquotes.pl?subject=Hepatolenticular degenerat

    32. ACP-ASIM Online - Medicine In Quotations Online
    Hemiplegia Hemodialysis Hemophilia Hemorrhoids HenochSchönlein purpura HeparinHepatitis hepatolenticular degeneration (Wilson disease) Herbs Hereditary
    http://www.acponline.org/cgi-bin/medquotes-subjects.pl?subjects=yes&letter=H

    33. Department Of Chemistry & Biochemistry At BYU - Clark J. Gubler - Publications
    DS Taylor, EJ Eichwald and MM Wintrobe, Chronic Manganese and Copper Poisoning inRats and Its Possible Relation to hepatolenticular degeneration in Man, Fed
    http://www.chem.byu.edu/emeritus/cjgubler/publications.html
    Selected Publications by Clark J. Gubler
    1. C.J. Gubler and J.E. Greaves, "The Cystine Content of Wheat and Its Quantitative Determination," Food Research 2. C.J. Gubler and C.L.A. Schmidt, "The Effect of Certain Salts on the Dissociation of Hemoglobin," Arch. Biochem. 3. C.J. Gubler, G.E. Cartwright and M.M. Wintrobe, "The Effect of Pyridoxine Deficiency on the Absorption of Iron by the Rat," J. Biol. Chem. 4. C.J. Gubler, G.E. Cartwright and M.M. Wintrobe, "The Anemia of Infection. X. The Effect of Infection on the Absorption and Storage of Iron by the Rat," J. Biol. Chem. 5. C.J. Gubler, G.E. Cartwright and M.M. Wintrobe, "The Anemia of Infection. XI. The Effect of Turpentine and Cobalt on the Absorption of Iron by the Rat," J. Biol. Chem.

    34. WILSON’S DISEASE
    Best viewed in Microsoft Explorer. WILSON’S DISEASE. (hepatolenticular degeneration).Anne Cording, Kellie Chase, Rachel Hasser. FScN 5623 Minavit Project.
    http://courses.che.umn.edu/02fscn5622-1s/02projects/Wilson's/wilsons_disease.htm
    Best viewed in Microsoft Explorer WILSON’S DISEASE (Hepatolenticular Degeneration) FScN 5623- Minavit Project Wilson’s Disease: Wilson’s disease (WD) is an inherited, autosomal recessive disorder that results in copper accumulation in the tissues of the body. Unlike other genetic disorders, WD is treatable. Approximately 1 in every 40,000 people is born with this disorder (1). WD is due to a defect on chromosome 13 of the ATP7B gene that expresses a membrane-bound copper-binding protein in the liver (6). Without this copper transporting ATPase, copper accumulates in the body, but especially in the liver causing cirrhosis and hepatitis; it also affects the brain. Copper deposits in the brain lead to damage of the basal ganglia and other nervous tissue and can cause loose movement coordination, trembling, and slurred speech (1). Psychiatric presentation involves behavioral abnormalities, like problems with temperament (1). Screening: Usually patients are diagnosed with WD when they start to present neuropsychiatric disorders or complications due to chronic liver disease (hepatitis, cirrhosis, or liver failure) (3). Patients usually present with symptoms between the ages of 10 and 40 (1). Copper Urine Concentrations g. Normal copper urine concentrations are between 20 and 50

    35. FScN 5622 Home Page
    Supplementation in the Treatment of Diabetes Stefanie Fila RD, Amy Gray, andSara Sjoberg Wilson's Disease (hepatolenticular degeneration) - Anne Cording
    http://courses.che.umn.edu/02fscn5622-1s/
    Vitamin and Mineral Metabolism
    FScN 5622
    (previously FScN 5623) Professor: Dr. Dan Gallaher
    Department of Food Science and Nutrition
    169 FScN
    2002 Projects DMT-1: An Iron Transporter - Sarah Adkins and Michelle McKean HFE: Tales from the Crypt - Melissa Perry, Hannah Olson, and Brittany Dokter Homocysteine - Katie Queenan, Allison Heinzeller, and Maia Dock
    Methylenetetrahydrofolate Reductase and Neural Tube Defects
    Pamela Carr, Mindi Khan and Dana Wells X-Linked Hypophosphatemia - Rebecca Blake, Michael Croy, and Brian Barrows Chromium Supplementation in the Treatment of Diabetes - Stefanie Fila RD, Amy Gray, and Sara Sjoberg
    Wilson's Disease (Hepatolenticular Degeneration)
    - Anne Cording, Kellie Chase and Rachel Hasser 2001 Projects Therapeutic Role of Antifolates - Andrea Plate, Melissa Lee and Kathy Nyquist The Incidence of Hyperhomocysteinemia in End Stage Renal Disease and the Potential for Treatment with Folate, Vitamins B12 and B6 - Jocelyn Mathern and Kimberly Morgan The Effect of Magnesium on Cardiac Arrythmias - Hasan Albasan, Melanie Praught, Steve Dandrea

    36. ICD-9-CM Disease Index: W
    Wesselsbron fever 066.3; West African fever 084.8; West Nile fever 066.3;WestphalStrumpell syndrome (hepatolenticular degeneration) 275.1;
    http://www.cpmc.columbia.edu/homepages/hripcsa/icd9/2indexw.html
    ICD-9-CM Index to Diseases: W
    • Waardenburg's syndrome 756.89
      • meaning ptosis-epicanthus 270.2
    • Waardenburg-Klein syndrome (ptosis-epicanthus) 270.2
    • Wagner's disease (colloid milium) 709.3
    • Wagner (-Unverricht) syndrome (dermatomyositis) 710.3
    • Waiting list, person on V63.2
      • undergoing social agency investigation V63.8
    • Wakefulness disorder (see also Hypersomnia) 780.54
      • nonorganic origin 307.43
    • Waldenstrom's
      • disease (osteochondrosis, capital femoral) 732.1
      • hepatitis (lupoid hepatitis) 571.49
      • hypergammaglobulinemia 273.0
      • macroglobulinemia 273.3
      • purpura, hypergammaglobulinemic 273.0
      • syndrome (macroglobulinemia) 273.3
    • Waldenstrom-Kjellberg syndrome (sideropenic dysphagia) 280.8
    • Walking
      • difficulty 719.7
      • psychogenic 307.9
      • sleep 307.46
      • hysterical 300.13
    • Wall, abdominal=see condition
    • Wallenberg's syndrome (posterior inferior cerebellar artery) (see also Disease, cerebrovascular, acute) 436
    • Wallgren's
      • disease (obstruction of splenic vein with collateral circulation) 459.89
      • meningitis (see also Meningitis, aseptic) 047.9
    • Wandering
      • acetabulum 736.39

    37. ICD-9-CM From Code 330
    Excludes hepatolenticular degeneration (275.1) multiple sclerosis (340);other demyelinating diseases of central nervous system (341.0341.9).
    http://www.cpmc.columbia.edu/homepages/hripcsa/icd9/1tabular330.html
    HEREDITARY AND DEGENERATIVE DISEASES OF THE CENTRAL NERVOUS SYSTEM (330-337)
    • Excludes: hepatolenticular degeneration (275.1)
      • multiple sclerosis (340)
      • other demyelinating diseases of central nervous system (341.0-341.9)
    • 330 Cerebral degenerations usually manifest in childhood
      • Use additional code to identify associated mental retardation
      • 330.0 Leukodystrophy
        • Krabbe's disease
        • Leukodystrophy:
          • NOS
          • globoid cell
          • metachromatic
          • sudanophilic
        • Pelizaeus-Merzbacher disease
        • Sulfatide lipidosis
      • 330.1 Cerebral lipidoses
        • Amaurotic (familial) idiocy
        • Disease:
          • Batten
          • Jansky-Bielschowsky
          • Kufs'
          • Spielmeyer-Vogt
          • Tay-Sachs
          • Gangliosidosis
        • 330.2 Cerebral degeneration in generalized lipidoses
          • Code first underlying disease, as:
            • Fabry's disease (272.7)
            • Gaucher's disease (272.7)
            • Niemann-Pick disease (272.7)
            • sphingolipidosis (272.7)
          • 330.3 Cerebral degeneration of childhood in other diseases classified elsewhere
            • Code first underlying disease, as:
              • Hunter's disease (277.5)
              • mucopolysaccharidosis (277.5)
            • 330.8 Other specified cerebral degenerations in childhood
              • Alpers' disease or gray-matter degeneration
              • Infantile necrotizing encephalomyelopathy
              • Leigh's disease
              • Subacute necrotizing encephalopathy or encephalomyelopathy
            • 330.9 Unspecified cerebral degeneration in childhood

    38. Florida State University College Of Medicine Digital Library
    CliniWeb Homepage (includes links to targeted PubMed MEDLINE searches)hepatolenticular degeneration List of documents. Pediatrics
    http://fsumed-dl.slis.ua.edu/clinical/gastroenterology/hepatic/hepatic/storagedi
    Clinical Resources by Topic: Gastroenterology
    Wilson Disease Clinical Resources
    Pediatrics Radiology Pathology Genetics ... Miscellaneous Resources See also:

    39. Wilson Disease
    Browse forwards .. Menu. Wilson Disease. Synonyms hepatolenticular degenerationMcKusick No. 27790. 1. Physical. KayserFleischer rings. 2. Neurological.
    http://oxmedinfo.jr2.ox.ac.uk/Pathway/Disease/82487.htm
    Metabolic pathway illustrated Browse backwards Browse forwards Menu
    Wilson Disease Synonyms Hepatolenticular Degeneration McKusick No.
    1. Physical
      Kayser-Fleischer rings.
    2. Neurological
    3. Gastrointestinal
    4. Renal
    5. Bone
    6. Comment
      Heterogeneity in time of onset and severity of symptoms. Generally patients present with either liver disease or neurological symptoms.
    7. Haematological
    8. Biochemical
      Low serum caeruloplasmin, low serum copper. Increased urinary copper. Elevated copper in liver. Enzyme EC number
    ................Diagnostics and therapy................
    Treatment
      D-Penicillamine or if this results in undesirable side effects, then triethylene tetramine (Trientine) is substituted.
    Carrier detection
      Possible in some families.
    Prenatal diagnosis
      Chromosome 13
    Expand periodical title abbreviations
      MBID (1989) Chapter 54
    This page last modified 11 Feb 1996.
    Pathway Webmaster, Oxford, UK

    40. Disease Index : H
    Hartnup disease Hawkinsinuria Hepatic fructokinase deficiency hepatolenticular degenerationHexosaminidase A deficiency HFI HGPRT deficiency HHH Syndrome HHHS
    http://oxmedinfo.jr2.ox.ac.uk/Pathway/Miscell/103984.htm
    A B C D ... Metabolic charts
    Disease index : H Hartnup disease
    Hawkinsinuria

    Hepatic fructokinase deficiency

    Hepatolenticular degeneration
    ...
    Hypoxanthine guanine
    This page last modified 11 Feb 1996.
    Pathway Webmaster, Oxford, UK

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