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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. Wilson Disease
Wilson disease, also known as hepatolenticular degeneration, is a rare inherited systemic Wilson s disease, also called hepatolenticular degeneration,
http://www.health-nexus.com/wilson_disease.htm
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Wilson Disease
Wilson Disease and Menkes Disease Progress in normal and disordered copper homeostasis, wilson disease, menkes disease, aceruloplasminemia, basic and clinical research. 8th International Conference, April 16 18, 2001.
WE MOVE - Wilson Disease WE MOVE - Wilson Disease ... Wilson disease, also known as hepatolenticular degeneration, is a rare inherited systemic disorder of copper metabolism. In patients with this disease, copper initially ...
Wilson's Disease Association International volunteer organization striving to promote the well-being of patients, their families, and friends.
Wilson disease Wilson disease. Disease type: Copper transport disorder Chromosomes: 13 Aetiology Wilsons...Sarkar B. (1997) Copper transport and its alterations in Menkes and Wilson disease. Biochimica et Biophysica acta. 1360, 3-16. See also: Menkes disease...

62. Arch Dermatol -- Abstract: Skin Lesions Induced By Penicillamine. Occurrence In
A 41year-old patient with hepatolenticular degeneration (Wilson disease), who had been treated for 15 years with penicillamine, developed small white
http://archderm.ama-assn.org/cgi/content/abstract/112/9/1267
Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress
Vol. 112 No. 9, September 1976 Featured Link E-mail Alerts ARTICLE Article Options Send to a Friend Readers Reply Submit a reply Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager PubMed citation Articles in PubMed by Greer KE Richardson DR Contact me when this article is cited
Skin lesions induced by penicillamine. Occurrence in a patient with hepatolenticular degeneration (Wilson Disease)
K. E. Greer, F. C. Askew and D. R. Richardson
A 41-year-old patient with hepatolenticular degeneration (Wilson disease), who had been treated for 15 years with penicillamine, developed small white papules at sites of venipuncture in the antecubital fossae and at surgical suture sites. Histologically, these papules showed focal areas of connective tissue degeneration in the dermis, but there was no evidence of

63. Hepatolenticular Degeneration - BlueRider.com
hepatolenticular degeneration listen domain availability. Dictionary and Thesaurus entries for. hepatolenticular degeneration. Your search results
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Web bluerider.com 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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64. Wilson's Disease (www.whonamedit.com)
hepatocerebral degeneration, hepatocerebral dystrophy, hepatolenticular degeneration, Morbus Wilson, neurohepatic degeneration, progressive lenticular
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

65. Samuel Alexander Kinnier Wilson (www.whonamedit.com)
which was also known as hepatolenticular degeneration . Wilson about his opinion on the essential criteria of hepatolenticular degeneration .
http://www.whonamedit.com/doctor.cfm/1711.html

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Whonamedit.com does not give medical advice.
This survey of medical eponyms and the persons behind them is meant as a general interest site only. No information found here must under any circumstances be used for medical purposes, diagnostically, therapeutically or otherwise. If you, or anybody close to you, is affected, or believe to be affected, by any condition mentioned here: see a doctor.
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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.

66. Wilson S Disease
Alternative Names. hepatolenticular degeneration. Causes. Wilson s disease is a rare inherited disorder. If both parents carry an abnormal gene for Wilson s
http://www.ehendrick.org/healthy/000785.htm
Injury Disease Nutrition Poison ... Prevention
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
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. If not caught and treated early, Wilson's disease is fatal. 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.

67. WHAT IS WILSON DISEASE
Wilsons disease (hepatolenticular degeneration) Hemochromatosis. These two diseases connected with the liver can cause cirrhosis – and may also be
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.

68. Wilson's Disease
This causes a variety of effects, including liver disease and damage to the nervous system. Alternative Names. hepatolenticular degeneration
http://www.healthscout.com/ency/1/000785.html
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Wilson's disease
Injury Disease Nutrition Poison ... Prevention
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. If not caught and treated early, Wilson's disease is fatal.

69. Neuropathology Mini-Course
chorea and Wilson s disease, or hepatolenticular degeneration. Huntington s disease is characterized by degeneration of the caudate nucleus,
http://www.pathology.vcu.edu/WirSelfInst/BASALGANG.html
Home About Us Site Directory Clinical Services ... Neuropathology Mini-Course NEUROPATHOLOGY MINI-COURSE
Presented by William I. Rosenblum, MD CHAPTER 6 DISEASES OF THE BASAL GANGLIA AND SUBSTANTIA NIGRA PRETEST: Answers will be found in the text of this chapter
  • What neurotransmitter is produced in the substantia nigra and where does it go? What color is the normal substantia nigra and why? What happens to the subst nigra grossly and microscopically in Parkinsons disease? What is the inclusion body that characterizes idiopathic Parkinsons and what peptide does it contain? Parkinsons disease maybe accompanied by dementia. What other disease of basal ganglia is characterized by dementia and is hereditary? In the later disease what is the key genetic feature? The key gross change? What disease is associated with excessive copper deposition in brain, eye and liver?
  • In the diseases discussed here, the primary pathology within the brain is located either in the basal ganglia or in the substantia nigra. The student should review the anatomy of these structures. The diseases involving the basal ganglia are Huntington's chorea and Wilson's disease, or hepatolenticular degeneration. The disease involving the substantia nigra is Parkinson's disease. The reason for grouping the latter disease together with the former is simple: the neuronal cell bodies in the substantia nigra are connected via their axons with cell bodies in the basal ganglia. The neurotransmitter released at the synapse in the basal ganglia is DOPamine (dihydroxyphenylethylamine), a catecholamine closely related to norepinephrine, and synthesized in the neurons of the substantia nigra. The transmitter travels in the axons to the basal ganglia. Hence destruction of the nigra results in depletion of DOPamine in the basal ganglia.

    70. British Anti-Lewisite :: References
    of brain and liver in the normal and in hepatolenticular degeneration. of BAL (2,3dimercaptopropanol) on hepatolenticular degeneration (Wilson’s
    http://www.chm.bris.ac.uk/motm/bal/references.html
    British anti-Lewisite By Domingo Tabangcura, Jr. and G. Patrick Daubert, MD Introduction Development Biochemistry Pharmacology ... References
    References
  • Wolf, H.W., R, Rubber: A Story of Glory and Greed. 1936, New York: Covici, Friede. Ord, M. and L. Stocken, A contribution of chemical defence in World War II. Trends Biochem Sci, 2000. : p. 253-6. Peters, R., L. Stocken, and R. Thompson, British anti-Lewisite (BAL). Nature, 1945. : p. 616-9. Subramanian, I., Z. Vanek, and J. Bronstein, Diagnosis and treatment of Wilson's disease. Curr Neurol Neurosci Rep, 2002. (4): p. 317-23. Cumings, J., The copper and iron content of brain and liver in the normal and in hepatolenticular degeneration. Brain, 1948. : p. 410-5. Mandelbrote, B., et al., Studies on copper metabolism in demyelinating diseases of the central nervous system. Brain, 1948. : p. 212-28. Denny-Brown, D. and H. Porter, The effect of BAL (2,3-dimercaptopropanol) on hepatolenticular degeneration (Wilson’s disease). Proc Am Neurol Assoc, 1951(79-84). Vilensky, J. and K. Redman
  • 71. Information On Copper; Product Review Where To Buy
    Those who suffer from Wilson’s disease (hepatolenticular degeneration), liver failure or kidney failure should not use copper supplements without strict
    http://www.allstarhealth.com/lj_c/Copper.htm
    YOUR CART Items Savings Total
    Copper
    What is Copper?
    Copper is an essential mineral necessary for human health. Copper is involved in development during infancy, the formation of collagen, heart contraction, glucose metabolism, cholesterol metabolism, brain development and proper immune function, among many other biological processes. Copper is also thought to have antioxidant and anti-inflammatory properties. The mineral can be found in oysters, nuts, seeds, legumes, and the bran and germ of whole grains.
    Deficiency and Toxicity
    Copper deficiency is characterized by several unpleasant physical and neurological symptoms. Low levels of copper in the body are associated with anemia, neutropenia, and osteoporosis.
    Copper
    is extremely toxic if consumed in large amounts (grams), which may result in coma, lung collapse, other dangerous symptoms, and death.
    Health Benefits
    While necessary for good overall health, copper may have additional benefits.
    Copper
    may help ward off some cancers, heart disease, high blood pressure, and keep cholesterol levels healthy.
    Copper
    constitutes part of the hair and skin pigment melanin, and may help keep skin healthy and hair color vibrant.

    72. Wilson's Disease (hepatolenticular Degeneration)
    Wilson s disease (hepatolenticular degeneration). A recessive genetic disease in which affected individuals can t process and excrete copper properly;
    http://www.cartage.org.lb/en/themes/Reference/dictionary/Biologie/W/61.html
    Wilson's disease (hepatolenticular degeneration) 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.

    73. Dreddyclinic.com - Wilson S Disease
    Wilson s disease (hepatolenticular degeneration) delayed progression of age related macular degeneration and reduced the risk of loss of vision.
    http://www.dreddyclinic.com/findinformation/ww/wilsonsdisease.htm

    74. Indian Journal Of Medical Research: DNA Linkage Based Diagnosis Of Wilson Diseas
    caused by defects in ATP7B gene located in chromosome 13q14, and manifested as hepatolenticular degeneration as a result of accumulation of copper.
    http://www.findarticles.com/p/articles/mi_qa3867/is_200311/ai_n9325093
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    ABNF Journal, The AIDS Treatment News AMAA Journal ... View all titles in this topic Hot New Articles by Topic Automotive Sports Top Articles Ever by Topic Automotive Sports DNA linkage based diagnosis of Wilson disease in asymptomatic siblings Indian Journal of Medical Research Nov 2003 by Gupta, A Neogi, R Mukherjea, M Mukhopadhyay, A ... Et al
    Save a personal copy of this article and quickly find it again with Furl.net. It's free! Save it. Methods: Genomic DNA was prepared from the peripheral blood of the patients, siblings and his/her first degree relatives. The repeat-markers flanking WD locus were amplified by PCR using fluorescent labeled primers. Amplified DNA fragments were analyzed by polyacrylamide gel electrophoresis in ABI 377 DNA sequencing system. Genotypes of the samples were determined using Genescan software. Haplotypes were determined based on segregation of the alleles in the families under study. Results: Among 15 WD affected families with multiple children, 4 cases were identified where younger siblings shared same genotype as the patient at all three markers analyzed. Further, eight different haplotypes were detected in the four patients.

    75. OpenMED@NIC - Subject: Hepatolenticular Degeneration
    OpenMED is a discipline based Open Access International Archive. It accepts both published and unpublished documents having relevance to research in Medical
    http://openmed.nic.in/view/subjects/C06.552.413.html
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    Subject: Hepatolenticular Degeneration

    76. Entrez PubMed
    Female; Gene Therapy/methods; hepatolenticular degeneration/diagnosis*; hepatolenticular degeneration/mortality; hepatolenticular degeneration/therapy*
    http://arthritis-research.com/pubmed/11906637
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    Pediatr Transplant.

    77. Entrez PubMed
    Adolescent; Adult; Female; FollowUp Studies; hepatolenticular degeneration/drug therapy*; hepatolenticular degeneration/physiopathology; Humans
    http://arthritis-research.com/pubmed/8859064
    My NCBI [Sign In] [Register] All Databases ... Books Search PubMed Protein Nucleotide Structure Genome Books CancerChromosomes Conserved Domains 3D Domains Gene Genome Project GENSAT GEO Profiles GEO DataSets HomoloGene Journals MeSH NCBI Web Site NLM Catalog OMIA OMIM PMC PopSet Probe PubChem BioAssay PubChem Compound PubChem Substance SNP Taxonomy UniGene UniSTS for Limits Preview/Index History Clipboard Details About Entrez
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    Arch Neurol.

    78. WilsonÉÂ,´Î¥ì¥ó¥º³ËÊÑÀ­¾É Wilson's Disesase,hepatolenticular D
    hepatolenticular degeneration. Wilson?, Wilson s disesase,hepatolenticular degeneration
    http://akimichi.homeunix.net/~emile/aki/medical/liver/node65.html
    Next: ´ÎÀ­¥Ý¥ë¥Õ¥£¥ê¥ó¾É hepatic porphyria Up: Previous: ·ì¿§ÁǾÉ,¥Ø¥â¥¯¥í¥Þ¥È¡¼¥·¥¹,Å´²á¾ê¾É,ÀÄƼ¿§ÅüǢɠhemochromatosis

    WilsonÉÂ,´Î¥ì¥ó¥º³ËÊÑÀ­¾É Wilson's disesase,hepatolenticular degeneration
    ŵµò: STEPÂå¼Õ¡¦ÆâʬÈç1ÈÇ [ , p.101] ŵµò: MorningReport [ , p.226] , p.373] ŵµò: DiagnosisAndTreatmentInGastroenterology [ , p.544] ŵµò: NIM¾²½´ïɳØ4ÈÇ [ , p.528] , p.368] ŵµò: Harper [ , p.713] , p.171] Kayer-FleischerÎØ
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  • Ƽ¥­¥ì¡¼¥ÈºÞ¤Ç¤¢¤ë D-¥Ú¥Ë¥·¥é¥ß¥ó D-Penicillamine ¤òÅêÍ¿¤¹¤ë¡£

  • Akimichi Tatsukawa

    79. HealthCyberMap: Nutritional And Metabolic Diseases And Immunity Disorders
    Resource Title Wilson Disease (hepatolenticular degeneration) Type/ Category Fact Sheet Language en URI Launch in new window. Show/ Hide Details
    http://healthcybermap.semanticweb.org/bodyviewer/nutritional.asp?Page=2

    80. 1759
    18 CASE hepatolenticular degeneration CLLNICAL MANIFESTATION Objective 18 patients with hepatolenticular degeneration (HLD) have a variety of clinical
    http://www.unepsa.org/china/ab/1759.HTM
    18 CASE HEPATOLENTICULAR DEGENERATION CLLNICAL MANIFESTATION Wang FL, XU YH, Yu J Chongqing Children¡¯s Hospital, Chongqing, China Objective: 18 patients with hepatolenticular degeneration (HLD) have a variety of clinical manifestation, which can cause mistakes in diagnosis. Method: 18 patients with HLD were analyzed using statistic describe in this study. Results: The mean age was 10 2.5 years old. The primary symptoms showed predominately in central nervous system (41.1%) and digestive (33.3%) system. Above 60% of the patients have the symptoms in the both systems during the course of development. Limb tremor and liver damages were the dominant symptoms. Almost 100% patients with HLD showed the decreased absorbent light degree of serum copper oxidase and ceruloplasmin, increased 24 hour urine copper and K-Fleischer ring in the eyes. The treatment using D-penicillamine was effective for 61.6% of the patients with HLD. Conclusion: most children with HLD were school age. The clinical manifestation was predominately found in nervous and digestive system.The decreased 24 hour urine copper and K-Fletcher in the eyes were the common and characteristic manifestation.

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