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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

61. Entrez PubMed
Hemochromatosis/physiopathology; Hemochromatosis/therapy; hepatolenticular degeneration/diagnosis; hepatolenticular degeneration/genetics
http://www.comparative-hepatology.com/pubmed/9929706
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Adv Intern Med. 1999;44:91-116. Related Articles, Links
New knowledge of genetic pathogenesis of hemochromatosis and Wilson's disease. Bacon BR, Schilsky ML. Division of Gastroenterology and Hepatology, Saint Louis University School of Medicine, Missouri, USA.

62. WilsonÉÂ,´Î¥ì¥ó¥º³ËÊÑÀ­¾É Wilson's Disesase,hepatolenticular D
Wilson?, Wilson s disesase,hepatolenticular degeneration. next up previous contents Next ?
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    63. Heilpflanzen-Welt - Die Welt Der Heilpflanzen!
    Metabolic balances of copper in patients with hepatolenticular degeneration submitted to vegetarian and mixed diets. Canelas HM, de Jorge FB, Tognola WA.
    http://www.heilpflanzen-welt.de/science/1967/6055347.htm

    64. ICH Slides Collection
    Clinical Pictures hepatolenticular degeneration. Subjects, Search, ( Click on picture to enlarge ). DI.2, DI.3.
    http://www.lib.uct.ac.za/ich/slides/thumb.php3?subjid=108

    65. ACP Online - Medicine In Quotations Online
    Health Library Wilson s Disease continue searching the Healthwise Knowledgebase. Copper Overload, hepatolenticular degeneration Wilson s disease is a genetic disorder
    http://www.acponline.org/cgi-bin/medquotes.pl?subject=Hepatolenticular degenerat

    66. Macular Degeneration
    Heavy Menstruation Heavy Menstruation Hemolytic Anemia Hemorrhoids Hepatitis hepatolenticular degeneration Herniated Disc Herpes (Genital) Herpes Simplex 1
    http://www.thenewhealthfind.com/health/macular degeneration
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          Directory   Home: Health: Conditions and Diseases: Eye Disorders: Macular Degeneration (32) Organizations (4) See Also: Health: Medicine: Medical Specialties: Ophthalmology Health: Senses: Vision Brian's Eye Story - A personal encounter. Contains photographs, new research, low vision aids, links, personal story and home eye tests. Macular Degeneration Partnership - Resources and information. Coalition of patients, families and leaders in the fields of vision and aging. The Macular Degeneration Network - A large-type educational and service oriented website for patients and the health professionals who care for them. Duke University Eye Center - Macular
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    67. Herniated Disc
    Menstruation Heavy Menstruation Hemolytic Anemia Hemorrhoids Hepatitis hepatolenticular degeneration Herniated Disc Herpes (Genital) Herpes Simplex 1 (Cold
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    68. Wilson's Disease Hub
    Wilson s Disease (hepatolenticular degeneration) by Diane W Cox, PhD, University of Alberta; Eve A Roberts, MD, University of Toronto.
    http://www.genomelink.org/wilson/
    Wilson's disease is an inherited disorder in which excessive amounts of copper accumulate in the body. The liver of a person who has Wilson's disease does not release copper into bile as it should. Symptoms usually appear between the ages of 6 and 20 years, but can begin as late as age 40. The primary consequence for approximately 40 percent of patients with Wilson's is liver disease. In other patients the first symptoms are either neurological or psychiatric or both, and include tremor, rigidity, drooling, difficulty with speech, abrupt personality change, grossly inappropriate behavior and unexplicable deterioration of school work, neurosis or psychosis. If not treated, Wilson's disease can cause severe brain damage, liver failure, and death. Wilson's Disease by the National Institute of Diabetes and Digestive and Kidney Diseases Wilson's Disease by Vanderbilt University Medical Center NINDS Wilson's Disease Information Page - by The National Institute of Neurological Disorders and Stroke. Wilson's Disease by the National Center for Biotechnology Information (NCBI) Wilson's Disease by University of Michigan Health System Hepatocerebral Degeneration - by Northwestern University Medical School.

    69. World J Gastroenterol
    MS, Zhang Z, Wu JX, Li F, Xue BC, Yang RM.Comparison of long lasting therapeutic effects between succimer and penicillamine on hepatolenticular degeneration.
    http://www.wjgnet.com/1007-9327/abstract_en.asp?f=530&v=4

    70. Hepatolenticular Degeneration - BlueRider.com
    hepatolenticular degeneration listen domain availability, Warning mysql_db_query 32 hepatolenticular degeneration. Your search results Warning
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    hepatolenticular degeneration [n] a rare inherited disorder of copper metabolism; copper accumulates in the liver and then in the red blood cells and brain Synonyms: wilson's_disease See Also:
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    71. ÎÞ±êÌâÎĵµ
    MR Imaging of hepatolenticular degeneration in Children GAO Yu, FAN Guoping, FENG Bin, et al (Department of Radiology, The Affiliated Xinhua Hospital of
    http://www.cjmit.com/HTML/Journals/2002-01/0025.htm
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    R445.2£» R596.1 [ÎÄÏ×±êʶÂë] A [ÎÄÕ±àºÅ] 1003-3289£¨2002£©01-0025-03
    MR Imaging of Hepatolenticular Degeneration in Children
    GAO Yu, FAN Guo-ping, FENG Bin, et al
    (Department of Radiology, The Affiliated Xinhua Hospital of Shanghai Second Medical University, Shanghai 200092, China)
    [Abstract] Objective To evaluate MRI in diagnosing hepatolenticular degeneration (HLD) in children. Methods Pretreatment brain and abdomen MRI were performed in 40 children with HLD. 12 patients who were treated were followed. Results Abnormal signals on brain MRI were seen in 23 cases, nodular abnormal signs on liver were seen in 16 cases. On the follow-up MRI of 12 patients, brain lesions of 7 patients shrunk or disappeared, and liver lesions of 6 patients disappeared. Conclusion MRI is of great significance in making diagnosis and prognosis of HLD.

    72. Wilson Disease --  Britannica Concise Encyclopedia Online Article
    or hepatolenticular degeneration. Recessive hereditary defect (see recessiveness) that impairs one s ability to metabolize copper.
    http://www.britannica.com/ebc/article?eu=408093&query=nageoires impaires&ct=

    73. Entrez PubMed
    Inherited copper toxicosis in Bedlington terriers Wilson s disease (hepatolenticular degeneration). Owen CA Jr, Ludwig J. MeSH
    http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstra

    74. EMedicine - Wilson Disease : Article Excerpt By: Celia H Chang, MD
    Wilson Disease Wilson disease, or hepatolenticular degeneration, is a neurodegenerative disease of copper metabolism. In 1912, Wilson
    http://www.emedicine.com/neuro/byname/wilson-disease.htm
    (advertisement)
    Excerpt from Wilson Disease
    Synonyms, Key Words, and Related Terms: Wilson’s disease, hepatolenticular degeneration, liver cirrhosis, copper overload, Kayser-Fleischer rings
    Please click here to view the full topic text: Wilson Disease
    Background: Wilson disease, or hepatolenticular degeneration, is a neurodegenerative disease of copper metabolism. In 1912, Wilson first described it as a familial disorder associated with neurological symptoms and cirrhosis. In 1956, Walshe first treated patients with the chelating agent penicillamine. Although the animal models are not equivalents of Wilson disease, they are helpful in studying copper metabolism and potential treatments. The Bedlington terrier has an autosomal recessive inherited disease characterized by copper toxicosis (reported by Owen and Ludwig in 1982). The Bedlington terrier does not develop neurological symptoms, but the liver pathology is similar to that of Wilson disease. The Long Evans Cinnamon (LEC) rat and toxic milk mouse both develop autosomal recessive inherited diseases associated with copper overload. LEC rats are reported to develop neurological symptoms, although hemolysis and hepatitis are the usual presenting symptoms; however, the liver histologic findings in the LEC rats and toxic milk mice are substantially different from those of Wilson disease. Tetrathiomolybdate was developed initially to treat chronic nutritional copper poisoning in sheep and is now used for Wilson disease. The Menkes disease gene (

    75. 000785
    Alternative Names hepatolenticular degeneration. Causes, incidence, and risk factors Wilson s disease is a rare inherited disorder.
    http://www.besthealth.com/Health Encyclopedia/articles/000785

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    Wilson's disease
    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.
    Alternative Names: Hepatolenticular degeneration
    Causes, incidence, and risk factors: 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. 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.

    76. 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

    77. WHAT IS WILSON DISEASE
    Hepatitis C website. Wilsons disease (hepatolenticular degeneration) Hemochromatosis. WILSONS DISEASE (hepatolenticular degeneration) HEMOCHROMATOSIS.
    http://home3.inet.tele.dk/omni/wilson.htm
    Updated January 27 th The Danish Hepatitis C website Wilsons disease (Hepatolenticular Degeneration) Hemochromatosis CIRRHOSIS In cirrhosis where the cause is related to the overload of certain metals, the metal burden in the body can be diminished by bloodletting (as in hemochromatosis) or medications to cause increased urine excretion (as in Wilson disease).
    Please see our comprehensive pages about cirrhosis
    WILSONS DISEASE (Hepatolenticular Degeneration) HEMOCHROMATOSIS WHAT IS WILSON DISEASE? Wilson disease is a relatively rare hereditary condition in which excessive amounts of copper accumulate in the body. (In Denmark approx. 50 people suffer from this liver disease). Recent data suggests the prevalence of the disorder to be about 1 in 30,000 worldwide with a carrier frequency of about 1 in 90. The disease is caused by the inheritance of a mutation on chromosome 13. The mutation prevents the body from eliminating excess copper. Defective mobilization of copper from hepatocellular lysosomes for excretion, via the bile, appears to underlie the pathogenesis of the disease. Ceruloplasmin is the protein that binds and removes excess copper and its levels are greatly reduced in this disorder. Neurological damage primarily occurs in the putamen and globus pallidus, collectively known as the lenticular nucleus.

    78. Samuel Alexander Kinnier Wilson (www.whonamedit.com)
    Following his exposition his name became attached to the disorder, which was also known as hepatolenticular degeneration . Wilson
    http://www.whonamedit.com/doctor.cfm/1711.html

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    Whonamedit.com does not give medical advice.
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    Samuel Alexander Kinnier Wilson
    British neurologist, born December 6, 1878, Cedarville, New Jersey, U.S.A.; died May 12, 1937, London.
    Writers disagree about his year of birth. Some say 1877, and one says 1874. Can you help? Associated eponyms: Foville-Wilson syndrome Term used to describe the impairment of lateral convergence that is present in disseminated sclerosis. Mallory's bodies II Alcoholic hyaline bodies. Wilson's disease A chronic disease of brain and liver with progressive neurological dysfunction, due to a disturbance of copper metabolism. Wilson's pronator sign Extension of the arms above the head will result in the palms facing outward in Sydenham chorea due to pronation of the forearms.

    79. Wilson's Disease (www.whonamedit.com)
    Cerebral pseudosclerosis, degeneratio hepatolenticularis, hepatocerebral degeneration, hepatocerebral dystrophy, hepatolenticular degeneration, Morbus Wilson
    http://www.whonamedit.com/synd.cfm/1818.html

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    Whonamedit.com does not give medical advice.
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    Wilson's disease Also known as:
    Kinnier Wilson's disease
    Westphal's pseudosclerosis Westphal-Strümpell disease Westphal-Strümpell pseudosclerosis Westphal-Strümpell syndrome Wilson-Konovalov disease Synonyms: Cerebral pseudosclerosis, degeneratio hepatolenticularis, hepatocerebral degeneration, hepatocerebral dystrophy, hepatolenticular degeneration, Morbus Wilson, neurohepatic degeneration, progressive lenticular degeneration. Associated persons: N. V. Konovalov Ernst Adolf Gustav Gottfried von Strümpell Karl Friedrich Otto Westphal Samuel Alexander Kinnier Wilson Description: A chronic disease of brain and liver with progressive neurological dysfunction, due to a disturbance of copper metabolism. Low ceruloplasmine levels permit accumulation of copper in the brain, liver, kidneys, and cornea. It is characterized by progressive degeneration of the basal ganglia of the brain, a brownish ring (Kayser-Fleischer ring) at the outer margin of the cornea caused by deposition of copper in the Descemet membrane, cirrhosis of the liver, splenomegaly, tremor, muscular rigidity, involuntary movements, spastic contractures, psychic disturbances, and progressive weakness and emaciation.

    80. Réseau Canadien De Technologie 
    ....... Experimental Model for Wilson s Disease hepatolenticular degeneration. Experimental Model for Wilson s Disease hepatolenticular degeneration.
    http://data.ctn-rct.nrc-cnrc.gc.ca/ctn/CTNServlet?calyHandler=OfferingPageHandle

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