{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T23:10:49Z","timestamp":1787613049560,"version":"build-2736575974"},"reference-count":23,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2021,3,23]],"date-time":"2021-03-23T00:00:00Z","timestamp":1616457600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Molec Gen &amp; Gen Med"],"published-print":{"date-parts":[[2021,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Although enzyme replacement therapy with agalsidase beta resulted in a variety of clinical benefits, life\u2010long biweekly intravenous infusion may impact on patients\u2019 quality of life. Moreover, regular infusions are time\u2010consuming: although a stepwise shortening of infusion duration is allowed up to a minimum of 1.5\u00a0hr, in most centers it remains \u22653\u00a0hr, and no data exists about the safety and tolerability of agalsidase beta administration at maximum tolerated infusion rate.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this study, we reported our experience with a stepwise infusion rate escalation protocol developed in our center in a cohort of 53 Fabry patients (both already receiving and treatment\u2010na\u03aave), and explored factors predictive for the infusion rate increase tolerability.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fifty\u2010two patients (98%) reduced infusion duration \u22643\u00a0hr; of these, 38 (72%) even reached a duration \u22642\u00a0hr. We found a significant difference between the mean duration reached by already treated and na\u03aave patients (<jats:italic>p<\/jats:italic>\u00a0&lt;\u00a0.01). More severely affected patients (male patients and those with lower enzyme activity) received longer infusions for higher risk of infusion\u2010associated reactions (IARs). A significant correlation between anti\u2010agalsidase antibodies and IARs was found.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our infusion rate escalation protocol is safe and could improve patient compliance, satisfaction and quality of life.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mgg3.1659","type":"journal-article","created":{"date-parts":[[2021,3,23]],"date-time":"2021-03-23T12:42:07Z","timestamp":1616503327000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Stepwise shortening of agalsidase beta infusion duration in Fabry disease: Clinical experience with infusion rate escalation protocol"],"prefix":"10.1002","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5379-1104","authenticated-orcid":false,"given":"Eleonora","family":"Riccio","sequence":"first","affiliation":[{"name":"Institute for Biomedical Research and Innovation National Research Council of Italy Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario","family":"Zanfardino","sequence":"additional","affiliation":[{"name":"IRCCS SDN Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monica","family":"Franzese","sequence":"additional","affiliation":[{"name":"IRCCS SDN Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivana","family":"Capuano","sequence":"additional","affiliation":[{"name":"Department of Public Health, Chair of Nephrology University Federico II of Naples Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pasquale","family":"Buonanno","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Reproductive and Odontostomatological Sciences University of Naples Federico II Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucia","family":"Ferreri","sequence":"additional","affiliation":[{"name":"Department of Public Health, Chair of Nephrology University Federico II of Naples Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Amicone","sequence":"additional","affiliation":[{"name":"Department of Public Health, Chair of Nephrology University Federico II of Naples Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Pisani","sequence":"additional","affiliation":[{"name":"Department of Public Health, Chair of Nephrology University Federico II of Naples Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,3,23]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-146-2-200701160-00148"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1086\/345309"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2017.12.435"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200107053450102"},{"key":"e_1_2_10_6_1","volume-title":"Fabrazyme summary of product characteristics (SmPC)"},{"key":"e_1_2_10_7_1","unstructured":"Fabrazyme\u00ae Prescribing Information. 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