Linked Life Data

12402283

Source:http://linkedlifedata.com/resource/pubmed/id/12402283

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SubjectPredicateObjectContext
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pubmed-article:12402283pubmed:dateCreated2002-10-28lld:pubmed
pubmed-article:12402283pubmed:abstractTextFabry's disease is commonly associated with a painful, debilitating neuropathy. Characterization of the physiological abnormalities is an important step in evaluating response to specific therapies. Twenty-two patients with Fabry's disease, and with relatively preserved renal function, underwent conventional and near-nerve conduction studies, electromyography, sympathetic skin responses, and quantitative sensory testing (QST). Nerve conduction studies were mostly normal except for an increased frequency of median nerve entrapment at the wrist in 6 (27%) patients. Sympathetic skin responses were preserved in 19 of 20 (95%) of the patients. The QST showed increased or immeasurable cold and warm detection thresholds in patients, significantly different from controls (n = 28) in the hand (P < 0.001, P = 0.04, respectively) and foot (P < 0.001 for both). Cold thresholds were more often abnormal than were warm thresholds. Vibration thresholds were normal in the feet and, in some patients, elevated in the hand only, probably due to frequent median nerve entrapment at the wrist. Our findings suggest that the neuropathy of Fabry's disease is characterized by an increased prevalence of median nerve entrapment at the wrist and by thermal afferent fiber dysfunction in a length-dependent fashion, with greater impairment of cold than warm sensation.lld:pubmed
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pubmed-article:12402283pubmed:authorpubmed-author:QuirkJane MJMlld:pubmed
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pubmed-article:12402283pubmed:copyrightInfoCopyright 2002 Wiley Periodicals, Inc. Muscle Nerve 26: 622-629, 2002lld:pubmed
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pubmed-article:12402283pubmed:volume26lld:pubmed
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pubmed-article:12402283pubmed:pagination622-9lld:pubmed
pubmed-article:12402283pubmed:dateRevised2010-11-18lld:pubmed
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pubmed-article:12402283pubmed:year2002lld:pubmed
pubmed-article:12402283pubmed:articleTitlePhysiological characterization of neuropathy in Fabry's disease.lld:pubmed
pubmed-article:12402283pubmed:affiliationElectromyography Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bldg. 10, Rm 3D03, 9000 Rockville Pike, Bethesda, Maryland 20892-1260, USA. cluciano@rcm.upr.edulld:pubmed
pubmed-article:12402283pubmed:publicationTypeJournal Articlelld:pubmed
pubmed-article:12402283pubmed:publicationTypeResearch Support, U.S. Gov't, P.H.S.lld:pubmed
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