{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T14:43:41Z","timestamp":1789915421451,"version":"4.0.1"},"reference-count":24,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,10,30]],"date-time":"2017-10-30T00:00:00Z","timestamp":1509321600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Biogen\/Bioverativ"},{"DOI":"10.13039\/501100012112","name":"Sobi","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100012112","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2018,1]]},"abstract":"\n Introduction<\/jats:title>\n Joint arthropathy is the long\u2010term consequence of joint bleeding in people with severe haemophilia.<\/jats:p>\n <\/jats:sec>\n \n Aim<\/jats:title>\n \n This study assessed change in joint health over time in subjects receiving recombinant factor\n VIII<\/jats:styled-content>\n Fc fusion protein (\n rFVIIIF<\/jats:styled-content>\n c) prophylaxis.\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n ALONG<\/jats:styled-content>\n is the phase 3 pivotal study in which the benefit of\n rFVIIIF<\/jats:styled-content>\n c as a prophylactic treatment for bleeding control was shown in previously treated severe haemophilia patients \u226512\u00a0years of age (arm 1: 25\u201065\n IU<\/jats:styled-content>\n \/kg every 3\u20105 days, arm 2: 65\n IU<\/jats:styled-content>\n \/kg weekly and arm 3: episodic). After completing\n ALONG<\/jats:styled-content>\n , subjects had the option to enrol into the extension study (\n ASPIRE<\/jats:styled-content>\n ). This interim, post hoc analysis assessed changes in joint health over ~2.8\u00a0years in these patients.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Forty\u2010seven subjects had modified Haemophilia Joint Health Score (\n mHJHS<\/jats:styled-content>\n ) data at A\u2010\n LONG<\/jats:styled-content>\n baseline,\n ASPIRE<\/jats:styled-content>\n baseline and\n ASPIRE<\/jats:styled-content>\n Year 1 and Year 2. Compared with A\u2010\n LONG<\/jats:styled-content>\n baseline (23.4), mean improvement at\n ASPIRE<\/jats:styled-content>\n Year 2 was \u22124.1 (95% confidence interval [\n CI<\/jats:styled-content>\n ], \u22126.5, \u22121.8;\n P\u00a0<\/jats:italic>\n =\n \u00a0<\/jats:italic>\n .001). Regardless of prestudy treatment regimen, subjects showed continuous improvement in\n mHJHS<\/jats:styled-content>\n from A\u2010\n LONG<\/jats:styled-content>\n baseline through\n ASPIRE<\/jats:styled-content>\n Year 2 (prestudy prophylaxis: \u22122.4,\n P\u00a0<\/jats:italic>\n =\n \u00a0<\/jats:italic>\n .09; prestudy episodic treatment: \u22127.2,\n P\u00a0<\/jats:italic>\n =\n \u00a0<\/jats:italic>\n .003). Benefits were seen in subjects with target joints (\u22125.6,\n P<\/jats:italic>\n \u00a0=\u00a0.005) as well as those with severe arthropathy (\u22128.8,\n P<\/jats:italic>\n \u00a0=\u00a0.02). The\n mHJHS<\/jats:styled-content>\n components with the greatest improvement at\n ASPIRE<\/jats:styled-content>\n Year 2 were swelling (\u22121.4,\n P\u00a0<\/jats:italic>\n =\n \u00a0<\/jats:italic>\n .008), range of motion (\u22121.1,\n P\u00a0<\/jats:italic>\n =\n \u00a0<\/jats:italic>\n .03) and strength (\u22120.8,\n P\u00a0=\u00a0<\/jats:italic>\n .04).\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n \n Prophylaxis with\n rFVIIIF<\/jats:styled-content>\n c may improve joint health over time regardless of prestudy prophylaxis or episodic treatment regimens.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/hae.13353","type":"journal-article","created":{"date-parts":[[2017,10,30]],"date-time":"2017-10-30T01:12:47Z","timestamp":1509325967000},"page":"77-84","source":"Crossref","is-referenced-by-count":43,"title":["Improved joint health in subjects with severe haemophilia A treated prophylactically with recombinant factor\n VIII<\/scp>\n Fc fusion protein"],"prefix":"10.1111","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3038-2145","authenticated-orcid":false,"given":"J.","family":"Oldenburg","sequence":"first","affiliation":[{"name":"Institute of Experimental Haematology and Transfusion Medicine University Clinic Bonn Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9372-3184","authenticated-orcid":false,"given":"R.","family":"Kulkarni","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Human Development Michigan State University East Lansing MI USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5032-5020","authenticated-orcid":false,"given":"A.","family":"Srivastava","sequence":"additional","affiliation":[{"name":"Department of Haematology Christian Medical College Vellore Tamil Nadu India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. N.","family":"Mahlangu","sequence":"additional","affiliation":[{"name":"Haemophilia Comprehensive Care Centre Faculty of Health Sciences University of the Witwatersrand and NHLS Johannesburg South Africa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V. S.","family":"Blanchette","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Toronto and Division of Hematology\/Oncology Hospital for Sick Children University of Toronto Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Tsao","sequence":"additional","affiliation":[{"name":"Bioverativ Waltham MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Winding","sequence":"additional","affiliation":[{"name":"Sobi Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Dumont","sequence":"additional","affiliation":[{"name":"Bioverativ Waltham MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"Jain","sequence":"additional","affiliation":[{"name":"Bioverativ Waltham MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,10,30]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.2147\/JBM.S50644"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.15256\/joc.2011.1.2"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.seminhematol.2015.10.005"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-01-528414"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12539"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa067659"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-08-367813"},{"key":"e_1_2_8_9_1","first-page":"235","article-title":"Fc\u2010fusion proteins and FcRn: structural insights for longer lasting and more effective therapeutics","volume":"35","author":"Rath T","year":"2013","journal-title":"Curr Opin Biotechnol"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.12076"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-09-382846"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.biologicals.2015.05.012"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-10-529974"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.12911"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12766"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-12-470898"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.20353"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01312.x"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2004.00998.x"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.12672"},{"key":"e_1_2_8_21_1","unstructured":"PasiKJ PabingerI KerlinBA et\u00a0al.Long\u2010term Efficacy of Recombinant Factor VIII Fc Fusion Protein (rFVIIIFc) Prophylaxis in Paediatric Adolescent and Adult Subjects With Target Joints and Severe Haemophilia A. Presented at: the 9th Annual Congress of the European Association for Haemophilia and Allied Disorders (EAHAD) February 3\u20105 2016 Malmo Sweden 2016."},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.291.5503.484"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(93)92000-J"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1038\/nri2206"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1586\/ehm.12.27"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.13353","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.13353","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T13:17:20Z","timestamp":1695475040000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.13353"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,10,30]]},"references-count":24,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2018,1]]}},"alternative-id":["10.1111\/hae.13353"],"URL":"https:\/\/doi.org\/10.1111\/hae.13353","archive":["Portico"],"relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.732291460.793544799","asserted-by":"object"}]},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,10,30]]}}}