41. CAS Appendix I Subj Hdngs 30a-z Intestine, neoplasm * * Liver, disease * * * BuddChiari syndrome * * * Cirrhosis* * * Hepatitis * * * hepatolenticular degeneration * * * Jaundice * * * Liver http://www-sul.stanford.edu/depts/swain/casapp/sub8a.html

Chemical Abstracts Appendix I Subject Headings 30a - z A publication of the Chemical Abstracts Service published by the American Chemical Society, and reproduced here with permission from the Chemical Abstracts Service. General Subject Headings Menu Keyword Index Menu Main Menu 30. MEDICINE sections: a - z aa - al

42. Wilson Disease Disease Wilson disease, also known as hepatolenticular degeneration,is a rare inherited systemic disorder of copper metabolism. In http://www.health-nexus.com/wilson_disease.htm

Health-Nexus.Net Health-Nexus.Org The #1 Health information site Home ... Up Search Health-Nexus for: Match ALL words Match ANY word Email this page to a friend ! Post a question or comment on our Message Board Home Page Health Specialties Health News ... Alternative Health Options Substance Abuse Animal Health Search: Books Magazines Video Keywords: Find it Here 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...

43. Hepatolenticular Degeneration hepatolenticular degeneration. A System. A resource with information onover 4000 medical topics including hepatolenticular degeneration. http://www.bloodandmarrowtransplant.com/medical-terms/02086.htm

Hepatolenticular degeneration A Medical Encyclopedia Article provided by Maryland General Hospital A resource with information on over 4000 medical topics including: Hepatolenticular degeneration Previous Next

44. 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

45. Clinical Pictures HAY FEVER. HENLOCH SCHONLEIN (ANAPHALYCTOID) SYNDROME. HENLOCH SCHONLEIN PURPURASYNDROME. hepatolenticular degeneration. HERMAPHRODITISM. HERNIA. HERNIA INGUINAL. http://www.lib.uct.ac.za/ich/slides/slides.php3?searchfor='H%'

46. MEDLINEplus Medical Encyclopedia: Wilson Disease Alternative names Return to top hepatolenticular degeneration. DefinitionReturn to top An inherited disorder where there is excessive http://www.nlm.nih.gov/medlineplus/ency/article/000785.htm

Skip navigation Medical Encyclopedia Other encyclopedia topics: A-Ag Ah-Ap Aq-Az B-Bk ... Z Wilson disease Contents of this page: Illustrations Alternative names Definition Causes, incidence, and risk factors ... Prevention Illustrations Central nervous system Copper urine test Alternative names Return to top Hepatolenticular degeneration Definition Return to top 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. Causes, incidence, and risk factors Return to top 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

47. MEDLINEplus: Wilson's Disease Wilson's Disease (American Liver Foundation). Clinical Trials ClinicalTrials.govhepatolenticular degeneration (National Institutes of Health). http://www.nlm.nih.gov/medlineplus/wilsonsdisease.html

Skip navigation Other health topics: A B C D ... List of All Topics Wilson's Disease Contents of this page: From the NIH General/Overviews Nutrition Research ... Organizations Search MEDLINE for recent research articles on Wilson's Disease You may also be interested in these MEDLINEplus related pages: Brain and Nervous System Digestive System Genetics/Birth Defects From the National Institutes of Health Wilson's Disease (National Digestive Diseases Information Clearinghouse) Wilson's Disease (National Institute of Neurological Disorders and Stroke) General/Overviews About Wilson's Disease (Wilson's Disease Association) Wilson Disease (We Move) Wilson's Disease (American Liver Foundation) Clinical Trials ClinicalTrials.gov: Hepatolenticular Degeneration (National Institutes of Health) Nutrition Copper: Reducing It in Your Diet (Mayo Foundation for Medical Education and Research) Research Wilson's Disease (National Center for Biotechnology Information) Specific Conditions/Aspects Frequently Asked Questions (Wilson's Disease Association) Genetics and Inheritance of Wilson's Disease (Wilson's Disease Association) Journals/Newsletters WDA Newsletters (Wilson's Disease Association) Organizations National Institute of Diabetes and Digestive and Kidney Diseases National Institute of Neurological Disorders and Stroke Wilson's Disease Association International (Wilson's Disease Association) Health services in North Carolina Other states coming - learn more The primary NIH organization for research on Wilson's Disease is the

48. Zeal.com - United States - New - Personal - Health - Conditions & Illnesses - Di organdonor.org/sarah.html Recounts the story of Sarah Osborn who, diagnosed in 1980with hepatolenticular degeneration, received a successful liver transplant. http://www.zeal.com/category/preview.jhtml?cid=274796

49. Zeal.com Health Conditions Illnesses Brain Nervous System Central Nervous System Brain Diseases Basal Ganglia hepatolenticular degeneration@ Edit Remove; http://www.zeal.com/category/profile.jhtml?cid=274796

50. 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

Home List categories Eponyms A-Z Biographies by country ... Contact Samuel Alexander Kinnier Wilson British neurologist, born December 6, 1878, Cedarville, New Jersey, U.S.A.; died May 12, 1937, London. 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. Wilson's sign Eccentric pupil in neoencephalic disease. Biography: Samuel Alexander Kinnier Wilson was born in New Jersey, USA. His father was the Reverend James Kinnier Wilson, a native of Ireland and a Presbyterian priest who died when Samuel was but one year of age. The family then moved back to their home in Edinburgh. He was educated at George Watson's College, Edinburgh, where his exceptional language skill earned him several prizes for Greek and Latin. He subsequently paid his way through his medical studies at the University of Edinburgh by teaching these disciplines. After graduating M.B. from the University of Edinburgh in 1902 Wilson became house physician at the Royal Edinburgh Infirmary with Sir Byrom Bramwell (1847-1931), and it was here he gained his lifelong interest in neurology. In 1903 he gained his B.sc. in physiology with honours, and that year went to Paris to work with Pierre Marie (1853-1940), before working for one year with Joseph Babinski (1857-1932) at the Bicêtre. After a short visit to Leipzig he returned to London, where in 1904 he came to the National Hospital for Nervous Diseases, Queen Square. Commencing as a house physician, he became registrar and pathologist, and up to honorary physician. Here he spent most of his professional life, with a group of neurologists that included Sir William Richard Gowers (1845-1915), John Hughlings Jackson (1835-1911), Henry Charlton Bastian (1837-1915) and Sir Victor Alexander Haden Horsley (1857-1916).

51. 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

Home List categories Eponyms A-Z Biographies by country ... Contact 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. A rare, autosomal recessively inherited condition which most often presents between 10 and 30 years of age. Slightly more prevalent in males. Females more often present with hepatic failure, males more often with neurological symptoms. In group of Eastern Europe extraction, later onset. Frequency is 30-60 per one million. The faulty gene has been localized to chromosome 13.

52. P Comparison of long lasting therapeutic effects between succimer and penicillamineon hepatolenticular degeneration. hepatolenticular degeneration. http://www.wjgnet.com/1007-9327/4/530.htm

P.O.Box 2345, Beijing 100023,China World J Gastroenterol Email: wcjd@public.bta.net.cn WJG ISSN 1007-9327 CN 14-1219/ R http:// www.wjgnet.com 1998 by The WJG Press Comparison of long lasting therapeutic effects between succimer and penicillamine on hepatolenticular degeneration Ren MS, Zhang Z, Wu JX, Li F, Xue BC, Yang RM Subject headings hepatolenticular degeneration/drug therapy; succimer/therapeutic use; penicillamine/therapeutic use Ren MS, Zhang Z, Wu JX, Li F, Xue BC, Yang RM.Comparison of long lasting therapeutic effects between succimer and penicillamine on hepatolenticular degeneration.World J Gastroenterol, 1998;4(6):530-532 Abstract AIM To compare the long-term effect of succimer (Suc) with that of penicillamine (Pen) in treating hepatolenticular degeneration (HLD). METHODS One hundred and twenty patients with HLD were divided into 2 groups. Group A (n=60) received Suc 750mg, po. bid. Group B (n=60) received Pen 250mg, po. qid. The period of maintenance treatment varied from 6 months to 3 years, averaging 1.5 years. Symptoms and therapeutic effects were evaluated by modified Goldstein scale. RESULTS The total effectiveness of group A in two different periods of treatment were 80% and 85% respectively, higher than those of group B (58% and 59% respectively) (P

53. Wilson's Disease Wilson's disease Alternative Names hepatolenticular degeneration Definition Wilson'sdisease is an inherited inborn error of metabolism in which the body http://atoz.iqhealth.com/HealthAnswers/encyclopedia/HTMLfiles/420.html

Wilson's disease Alternative Names hepatolenticular degeneration Definition Wilson's disease is an inherited inborn error of metabolism in which the body cannot process copper. What is going on in the body? Wilson's disease is caused by a defect in an enzyme, or special protein. This enzyme normally helps the body get rid of extra copper. The amount of copper in a normal diet is more than the body needs. In people with Wilson's disease, the extra copper cannot be excreted from the body. The copper can damage the liver, brain, kidney, cornea, and other organs. What are the signs and symptoms of the disease? Wilson's disease may cause: anemia , or low red blood cell counts ataxia , or lack of coordination jaundice , or yellow eyes and skin kidney problems, which are usually minor loss of appetite psychiatric problems, such as anxiety, depression , personality changes, and psychosis slurred speech or inability to form words tremors, or involuntary rhythmic movements weakness and a general discomfort What are the causes and risks of the disease?

54. Liver Diseases viral Hepatitis B CDC. hepatolenticular degeneration Search PUBMEDfor hepatolenticular degeneration All Review Therapy Diagnosis; http://www.ohsu.edu/cliniweb/C6/C6.552.html

Liver Diseases Back to previous level Cholestasis, Intrahepatic [2 more specific term/s, 34 more link/s] Search PUBMED for Cholestasis, Intrahepatic: All Review Therapy Diagnosis ... Fatty Liver [2 more specific term/s, 6 more link/s] Search PUBMED for Fatty Liver: All Review Therapy Diagnosis ... Nonalcoholic Steatohepatitis in Obese Person - Histopathology: Virtual Hospital, U. of Iowa Hepatic Vein Thrombosis Search PUBMED for Hepatic Vein Thrombosis: All Review Therapy Diagnosis ... Intrahepatic Portal Vein Thrombosis, Pathologic Photo: Virtual Hospital, U. of Iowa Hepatic Veno-Occlusive Disease Search PUBMED for Hepatic Veno-Occlusive Disease: All Review Therapy Diagnosis ... Hepatitis [5 more specific term/s, 15 more link/s] Search PUBMED for Hepatitis: All Review Therapy Diagnosis ... viral Hepatitis B: CDC Hepatolenticular Degeneration Search PUBMED for Hepatolenticular Degeneration: All Review Therapy Diagnosis ... Wilsons disease: Vanderbilt U. Hepatomegaly Search PUBMED for Hepatomegaly: All Review Therapy Diagnosis ... Hepatomegaly: Med. Coll. of Wisconsin Hepatopulmonary Syndrome Search PUBMED for Hepatopulmonary Syndrome: All Review Therapy Diagnosis Hepatorenal Syndrome Search PUBMED for Hepatorenal Syndrome: All Review Therapy Diagnosis ... Hypertension, Portal

55. Basal Ganglia Diseases Coll. of Wisconsin. hepatolenticular degeneration Search PUBMED forhepatolenticular degeneration All Review Therapy Diagnosis; The http://www.ohsu.edu/cliniweb/C10/C10.228.140.79.html

Basal Ganglia Diseases Back to previous level Alzheimer Disease Search PUBMED for Alzheimer Disease: All Review Therapy Diagnosis ... Chorea [1 more specific term/s, 5 more link/s] Search PUBMED for Chorea: All Review Therapy Diagnosis ... Sydenhams Chorea: Vanderbilt U. Creutzfeldt-Jakob Syndrome Search PUBMED for Creutzfeldt-Jakob Syndrome: All Review Therapy Diagnosis ... Report of Meeting on Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: CDC Dyskinesia, Drug-Induced Search PUBMED for Dyskinesia, Drug-Induced: All Review Therapy Diagnosis ... Spontaneous Remission Rate of Tardive Dyskinesia: U. of Iowa Dystonia Musculorum Deformans Search PUBMED for Dystonia Musculorum Deformans: All Review Therapy Diagnosis ... DYSTONIA 3, TORSION, X-LINKED; DYT3 Hallervorden-Spatz Syndrome Search PUBMED for Hallervorden-Spatz Syndrome: All Review Therapy Diagnosis ... Hallervorden-Spatz disease: Med. Coll. of Wisconsin Hepatolenticular Degeneration Search PUBMED for Hepatolenticular Degeneration: All Review Therapy Diagnosis ... Wilsons disease: Vanderbilt U. Meige Syndrome Search PUBMED for Meige Syndrome: All Review Therapy Diagnosis ... Multiple System Atrophy [3 more specific term/s, 42 more link/s]

56. Vitamins Minerals Quasi-Vitamins Amino Acids Supplements We have of solution or cure of hepatolenticular degeneration and otherdiseases . Complete details hepatolenticular degeneration. Our top http://www.herbaladvisor.com/ailment/xq/asp/ailid.106/qx/almdetail.htm

Vitamins Minerals Quasi-Vitamins Amino Acids ... Cancer Products Brand Supplements Vitamins Minerals Amino Acids Ailments Herbs Articles Forums Member Login User Name Password New User? Register Here! Forgot Password? Click Here! Shopping Cart Item Qty Price Basket is empty Brand Names Source Naturals Enzymatic Therapy Eclectic Institute Inc. Raintree Nutrition Inc. Planetary Formulas Jarrow Formulas Inc Herb Pharm Marlyn/Naturally A To B Calm Alvita Teas American Health Amerifit (Formerly Strength Systems) Anabol Naturals Ark Naturals Arkopharma At Last Naturals (Formerly Alvin Last) Aura Cacia Auromere Auroshikha Floral Incense Auroshikha Flower Candles Avalon Organic Botanicals Bach Flower Essences Beano Beauty Without Cruelty Bee Pollen From England Bindi Biogenetics Bio-Strath / Anima Strath Biotec Biotherapies Body Tools Bodyzone Boiron Borlind Of Germany Champion Nutrition Chandrika Soap Childlife Chinese Imports Cholestaid Cyclone Cider Desert Essence Dolisos Dragon Eggs Earth Science Earthrise Ecodent (Formerly Merfluan) Emerald Forest Endurox Essiac International Etherium Technology Field Collector Series (Environmental Cassettes) Foodscience Of Vermont French Transit Ltd Fuchs Brushes Futurebiotics Ginsana/Pharmaton Great American Nutrition Green Ban Green Foods Corporation Habitat Health Products Han's Hawaiian Henna Natural Tattoo Kit Health And Body Fitness Health Concerns Health From The Sun Health Plus Herbalvedic Ayurvedic Formulas Herbavita Natural Hair Color Herbs For Kids Heritage Products Hobe Labs Holistic Horizons Holistic Products

57. UPMC/Radiology Residents Past Case Diagnosis hepatolenticular degeneration, or Wilson's disease. Hepatolenticulardegeneration, or Wilson's disease, is an automsomal recessive disorder. http://www.rad.upenn.edu/resmac/weekcase/Archives/Neuro1.html

T HE W EEK OF August 25, 1997 A SSOCIATED I MAGES: T HIS W EEK'S ... RCHIVES UPHS RADIOLOGY residency program Residents' Case of the Week H ISTORY: 17 year old boy with cirrhosis and first seizure. I D ISCUSSION: FINDINGS: Marked cortical thickening on plain film. Anterior soft tissue ulcer on MRI, with abnormal marrow signal on both T1 and T2. Diagnosis: Hepatolenticular degeneration, or Wilson's disease. Discussion: Abnormal low T2 signal intensity within the putamina and within white matter tracts, including the corpus callosum. Hepatolenticular degeneration, or Wilson's disease, is an automsomal recessive disorder. It has been traced to the long arm of chromosome 13 (13q14.3). This defect leads to insufficient copper excretion in bile, and resulting copper deposition incharacteristic locations, including the brain, eyes, and liver. Treatment is with copper chelation with D-penicilamine. Several studies have examined the MR imaging findings in Wilson's disease. [Saatci I et. al. Cranial MR findings in Wilson's disease, Acta Radiologica. 38(2):250-8, 1997 Mar.] [King AD et. al. Cranial MR imaging in Wilson's disease. AJR. American Journal of Roentgenology. 167(6):1579-84, 1996 Dec.] Atrophy is seen in almost all patients. A very common finding - not seen in this case - is that of symmetric, bilateral T2 hyperintensity within the putamina. The dorsal aspect of the pons, the midbrain, the thalami, and the caudate are also known sites of involvement. Cortical and subcortical white matter tracts may be involved. Paramagnetic effects of copper, with T1 shortening, may be seen in untreated patients.

58. Wilson Disease Wilson disease. Alternate Names hepatolenticular degeneration. Causesand Risks Wilson disease (hepatolenticular degeneration) is http://www.rwjuhh.net/Atoz/encyclopedia/article/000785.asp

For a complete list of hospital classes and events, click here to connect to HealthConnection Online Medical Encyclopedia Encyclopedia Disease W -> Wilson disease Wilson disease Alternate Names: Hepatolenticular degeneration Causes and Risks: 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 central nervous system (brain, spinal cord) are the most predominant, and the most dangerous, effects of the disorder.

59. Health Library - Wilson's Disease report. Synonyms. hepatolenticular degeneration; Lenticular Degeneration,Progressive. Disorder Subdivisions. None. General Discussion. http://health_info.nmh.org/Library/HealthGuide/IllnessConditions/topic.asp?hwid=

60. WHAT IS WILSON DISEASE Hepatitis C website. Wilsons disease (hepatolenticular degeneration) Hemochromatosis. WILSONSDISEASE (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.

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