{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T22:03:37Z","timestamp":1773007417709,"version":"3.50.1"},"reference-count":9,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2006,3,31]],"date-time":"2006-03-31T00:00:00Z","timestamp":1143763200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2006,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>We prospectively evaluated the effect of enzyme replacement therapy (ERT) on the intraepidermal nerve fiber density (IENFD) and thermal threshold in patients with Fabry disease, an X\u2010linked disorder associated with a painful small\u2010fiber neuropathy and decreased linear IENFD in a length\u2010dependent pattern. Twenty\u2010five hemizygous male patients with Fabry disease were enrolled in a 6\u2010month, randomized, placebo\u2010controlled ERT trial of 0.2 mg\/kg of \u03b1\u2010galactosidase A (agalsidase\u2010alfa) every 2 weeks followed by an additional 12 months of open\u2010label ERT for both populations. IENFD and thermal threshold were measured in the distal thigh at baseline, 6 months, and 18 months from initiation of the trial. We found no significant difference in IENFD between the treatment groups at 6 months. After an additional year of ERT, there was a significant reduction in IENFD in the patient group as a whole, attributable to the declining glomerular filtration rate. Thermal thresholds remained unchanged. We conclude that epidermal nerve fiber regeneration, as measured in the distal thigh, does not occur in this patient population after 12\u201318 months of ERT. Muscle Nerve, 2006<\/jats:p>","DOI":"10.1002\/mus.20550","type":"journal-article","created":{"date-parts":[[2006,3,31]],"date-time":"2006-03-31T13:48:12Z","timestamp":1143812892000},"page":"53-56","source":"Crossref","is-referenced-by-count":76,"title":["Enzyme replacement therapy and intraepidermal innervation density in Fabry disease"],"prefix":"10.1002","volume":"34","author":[{"given":"Raphael","family":"Schiffmann","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Hauer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Barbara","family":"Freeman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Markus","family":"Ries","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leland J. C.","family":"Scott","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Polydefkis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roscoe O.","family":"Brady","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Justin C.","family":"McArthur","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kathryn","family":"Wagner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,3,31]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM196705252762101"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1097\/00004691-200212000-00011"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000118207.84514.40"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-7216-9491-7.50037-5"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.10236"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.55.12.1513"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.285.21.2743"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.10497"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.52.6.1249"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmus.20550","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.20550","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,28]],"date-time":"2023-09-28T20:44:31Z","timestamp":1695933871000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.20550"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,3,31]]},"references-count":9,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2006,7]]}},"alternative-id":["10.1002\/mus.20550"],"URL":"https:\/\/doi.org\/10.1002\/mus.20550","archive":["Portico"],"relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.12664.470147","asserted-by":"object"}]},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,3,31]]}}}