{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,31]],"date-time":"2026-03-31T23:54:33Z","timestamp":1775001273745,"version":"3.50.1"},"reference-count":27,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2006,7,27]],"date-time":"2006-07-27T00:00:00Z","timestamp":1153958400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2006,10]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p><jats:italic>Aim<\/jats:italic> To report the effect of enzyme replacement therapy (ERT) in sympathetic skin responses (SSR) of patients with Fabry disease. <jats:italic>Patients and methods<\/jats:italic> Seven male patients were included in an open\u2010label protocol using agalsidase\u2010alfa, continued at regular intervals. Five patients completed 24 months of ERT and two of them completed 18 months. Two main measurements were performed at baseline, as well as 1 and 2 years after ERT: (1) a standard neurological examination (NE), with a detailed evaluation of the sensory perception of light touch, pinprick, cold, hot, and vibratory stimuli; (2) the SSR amplitudes. <jats:italic>Results<\/jats:italic> Although there were no significant differences between NE in this time period, all patients reported general improvement in their subjective reports of acroparaesthesia and sweating. Before starting ERT, the SSR amplitudes were either too small (3\/7 patients) or absent (4\/7 patients): the average (range) amplitude of 122 \u03bcV (0 through 492) was statistically smaller than that found in a control group, i.e. 1453.6 \u03bcV (619.7\u20132754) (<jats:italic>p<\/jats:italic>&lt;0.0001, <jats:italic>t<\/jats:italic>\u2010test). Mean \u00b1 SD SSR amplitude increased to 1088\u00b1 690 \u03bcV in the second year of ERT, reaching the range found in a normal control group (<jats:italic>p<\/jats:italic>=0.004). <jats:italic>Conclusion<\/jats:italic> ERT improved SSR continuously in Fabry patients in 2 years of observation. Although the mechanism of the SSR improvement is unknown, this response to ERT can be clinically significant if it reflects a normalization in sweating.<\/jats:p>","DOI":"10.1007\/s10545-006-0339-3","type":"journal-article","created":{"date-parts":[[2006,7,28]],"date-time":"2006-07-28T16:27:47Z","timestamp":1154104067000},"page":"653-659","source":"Crossref","is-referenced-by-count":17,"title":["Improvement of sympathetic skin responses under enzyme replacement therapy in Fabry disease"],"prefix":"10.1002","volume":"29","author":[{"given":"L. B.","family":"Jardim","sequence":"first","affiliation":[{"name":"Medical Genetics Service Hospital de Cl\u00ednicas de Porto Alegre Porto Alegre Brazil"},{"name":"Internal Medicine Department Universidade Federal do Rio Grande do Sul Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"I.","family":"Gomes","sequence":"additional","affiliation":[{"name":"Neurology Service Hospital de Cl\u00ednicas de Porto Alegre Porto Alegre Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C. B. O.","family":"Netto","sequence":"additional","affiliation":[{"name":"Medical Genetics Service Hospital de Cl\u00ednicas de Porto Alegre Porto Alegre Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D. B.","family":"Nora","sequence":"additional","affiliation":[{"name":"Neurology Service Hospital de Cl\u00ednicas de Porto Alegre Porto Alegre Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"U. 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