{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,6,18]],"date-time":"2024-06-18T21:12:29Z","timestamp":1718745149571},"reference-count":12,"publisher":"Wiley","issue":"S1","license":[{"start":{"date-parts":[[2009,6,28]],"date-time":"2009-06-28T00:00:00Z","timestamp":1246147200000},"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":[[2009,12]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Intravenous enzyme replacement therapy with recombinant human \u03b1\u2010<jats:sc>l<\/jats:sc>\u2010iduronidase (rhIDU) is used weekly to treat mucopolysaccharidosis (MPS) I. We tested continuous administration of rhIDU at two dosing levels (0.58 mg\/kg per week and 2 mg\/kg per week) in MPS I dogs, and compared the efficacy of continuous infusion with the clinically used 0.58 mg\/kg weekly three\u2010hour infusion. Peak plasma concentrations of rhIDU were much higher in weekly\u2010treated dogs (mean 256 units\/ml) than steady\u2010state concentrations in dogs treated with continuous infusion (mean 1.97 units\/ml at 0.58 mg\/kg per week; 8.44 units\/ml at 2 mg\/kg per week). Dogs receiving continuous IV rhIDU, even at a higher (2 mg\/kg per week) dose, had consistently lower iduronidase levels in tissues than dogs receiving a weekly (0.58 mg\/kg per week) dose. GAG storage was also less improved by continuous intravenous infusion. Adverse events were similar in all dosing groups. We found that continuous administration of 2 mg\/kg per week rhIDU to MPS I dogs was insufficient to achieve GAG storage reduction comparable to 0.58 mg\/kg weekly dosing.<\/jats:p>","DOI":"10.1007\/s10545-009-1198-5","type":"journal-article","created":{"date-parts":[[2009,6,27]],"date-time":"2009-06-27T09:21:13Z","timestamp":1246094473000},"page":"253-258","source":"Crossref","is-referenced-by-count":5,"title":["Continuous infusion of enzyme replacement therapy is inferior to weekly infusions in MPS I dogs"],"prefix":"10.1002","volume":"32","author":[{"given":"M. B.","family":"Passage","sequence":"first","affiliation":[{"name":"Los Angeles Biomedical Research Institute at Harbor UCLA 1124 W. Carson Street, E\u20104 Torrance CA 90502 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A. W.","family":"Krieger","sequence":"additional","affiliation":[{"name":"Los Angeles Biomedical Research Institute at Harbor UCLA 1124 W. Carson Street, E\u20104 Torrance CA 90502 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. C.","family":"Peinovich","sequence":"additional","affiliation":[{"name":"Los Angeles Biomedical Research Institute at Harbor UCLA 1124 W. Carson Street, E\u20104 Torrance CA 90502 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"Lester","sequence":"additional","affiliation":[{"name":"BioMarin Pharmaceutical Inc. Novato CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S. Q.","family":"Le","sequence":"additional","affiliation":[{"name":"Los Angeles Biomedical Research Institute at Harbor UCLA 1124 W. Carson Street, E\u20104 Torrance CA 90502 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P. I.","family":"Dickson","sequence":"additional","affiliation":[{"name":"Los Angeles Biomedical Research Institute at Harbor UCLA 1124 W. Carson Street, E\u20104 Torrance CA 90502 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E. D.","family":"Kakkis","sequence":"additional","affiliation":[{"name":"BioMarin Pharmaceutical Inc. Novato CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2009,6,28]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199105233242104"},{"key":"e_1_2_6_3_1","first-page":"2868","article-title":"Immune tolerance improves the efficacy of enzyme replacement therapy in the canine model of mucopolysaccharidosis I","volume":"118","author":"Dickson P","year":"2008","journal-title":"J Clin Invest"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200107053450102"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2008.10.009"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2005.tb02115.x"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1464-5491.2007.02311.x"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1006\/prep.1994.1035"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1006\/bmme.1996.0044"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200101183440304"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2006.02.035"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.gim.0000232477.37660.fb"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2006-2156"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-009-1198-5","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1007\/s10545-009-1198-5","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-009-1198-5","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T03:18:24Z","timestamp":1694747904000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-009-1198-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,6,28]]},"references-count":12,"journal-issue":{"issue":"S1","published-print":{"date-parts":[[2009,12]]}},"alternative-id":["10.1007\/s10545-009-1198-5"],"URL":"https:\/\/doi.org\/10.1007\/s10545-009-1198-5","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,6,28]]}}}