{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T05:00:22Z","timestamp":1791608422559,"version":"4.3.4"},"reference-count":30,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2012,9,7]],"date-time":"2012-09-07T00:00:00Z","timestamp":1346976000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2013,1]]},"abstract":"Summary<\/jats:title>Von Willebrand disease (VWD<\/jats:styled-content>) is an inherited bleeding disorder caused by the quantitative or qualitative deficiency of von Willebrand factor (VWF<\/jats:styled-content>). Replacement therapy with plasma\u2010derived VWF<\/jats:styled-content>\/factor VIII<\/jats:styled-content> (FVIII<\/jats:styled-content>) concentrates is required in patients unresponsive to desmopressin. To assess the efficacy, safety and ease of use of a new, volume\u2010reduced (VR<\/jats:styled-content>) formulation of VWF<\/jats:styled-content>\/FVIII<\/jats:styled-content> concentrate Haemate\u00ae<\/jats:sup> P in patients requiring treatment for bleeding or prophylaxis for recurrent bleeding or for invasive procedures. Pharmacoeconomic variables were also recorded. Data were analysed using descriptive statistics. This was a multicentre, prospective, observational study. Consecutively enrolled patients received Haemate<\/jats:styled-content>\u00ae<\/jats:styled-content><\/jats:sup> P<\/jats:styled-content> VR<\/jats:styled-content><\/jats:styled-content> according to their needs, and were followed for 24\u00a0months. Of the 121 patients enrolled, 25.6% had type 3 VWD<\/jats:styled-content> and more than 40% had severe disease. All patients were followed for 2\u00a0years, for a total of 521 visits. On\u2010demand treatment was given to 61.9% of patients, secondary long\u2010term prophylaxis to 25.6% and prophylaxis for surgery, dental or invasive procedures to 45.5%. The response to treatment was rated as good to excellent in >93\u201399% of interventions. The new formulation was well tolerated by all patients with no report of drug\u2010related adverse events. The switch to volume\u2010reduced Haemate\u00ae<\/jats:sup> P was easy to perform and infusion duration was decreased twofold compared with the previous formulation. Volume\u2010reduced Haemate\u00ae<\/jats:sup> P was at least as effective and well\u2010tolerated as the previous formulation.<\/jats:p>","DOI":"10.1111\/hae.12005","type":"journal-article","created":{"date-parts":[[2012,9,7]],"date-time":"2012-09-07T13:46:11Z","timestamp":1347025571000},"page":"82-88","source":"Crossref","is-referenced-by-count":31,"title":["Efficacy and safety during formulation switch of a pasteurized VWF\/FVIII concentrate: results from an Italian prospective observational study in patients with von Willebrand disease"],"prefix":"10.1111","volume":"19","author":[{"given":"G.","family":"Castaman","sequence":"first","affiliation":[{"name":"San Bortolo Hospital Vicenza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Coppola","sequence":"additional","affiliation":[{"name":"Federico II University Hospital Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Zanon","sequence":"additional","affiliation":[{"name":"University Hospital Padua Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Boeri","sequence":"additional","affiliation":[{"name":"Gaslini Hospital Genoa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Musso","sequence":"additional","affiliation":[{"name":"University Hospital Catania Catania Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Siragusa","sequence":"additional","affiliation":[{"name":"University Hospital Palermo Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. B.","family":"Federici","sequence":"additional","affiliation":[{"name":"IRCCS C\u00e0 Granda Maggiore Policlinico Hospital Foundation and Department of Internal Medicine AB Bonomi Hemophilia Thrombosis Center University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Mancuso","sequence":"additional","affiliation":[{"name":"Children's Hospital Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Barillari","sequence":"additional","affiliation":[{"name":"University Hospital Udine Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Biasoli","sequence":"additional","affiliation":[{"name":"Bufalini Hospital Cesena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Feola","sequence":"additional","affiliation":[{"name":"San Luca Hospital Vallo della Lucania Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Franchini","sequence":"additional","affiliation":[{"name":"Istituti Ospitalieri Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Moratelli","sequence":"additional","affiliation":[{"name":"University Hospital Ferrara Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Gamba","sequence":"additional","affiliation":[{"name":"San Matteo Hospital Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Schinco","sequence":"additional","affiliation":[{"name":"San Giovanni Battista Hospital Torino Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Valdr\u00e8","sequence":"additional","affiliation":[{"name":"S. Orsola\u2010Malpighi Hospital Bologna Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Dragani","sequence":"additional","affiliation":[{"name":"Spirito Santo Hospital Pescara Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Mazzucconi","sequence":"additional","affiliation":[{"name":"Umberto I Hospital Roma Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Tagliaferri","sequence":"additional","affiliation":[{"name":"University Hospital Parma Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Morfini","sequence":"additional","affiliation":[{"name":"Careggi Hospital Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2012,9,7]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2002.00672.x"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra040403"},{"key":"e_1_2_7_4_1","first-page":"94","article-title":"Von Willebrand's disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment","volume":"88","author":"Castaman G","year":"2003","journal-title":"Haematologica"},{"key":"e_1_2_7_5_1","first-page":"117","article-title":"Evidence\u2010based recommendations on the treatment of von Willebrand disease in Italy","volume":"7","author":"Mannucci PM","year":"2009","journal-title":"Blood Transfus"},{"issue":"2","key":"e_1_2_7_6_1","first-page":"S33","article-title":"Surgical prophylaxis in von Willebrand's disease: a difficult balance to manage","volume":"6","author":"Franchini M","year":"2008","journal-title":"Blood Transfus"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01847.x"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(09)70152-5"},{"key":"e_1_2_7_9_1","unstructured":"SPC of Haemate\u00aeP. Available online from URL:http:\/\/www.cslbehring.it\/docs\/229\/203\/RCP_Haemate%20P 0.pdf. Accessed January 21 2012."},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.11124"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.01847.x"},{"key":"e_1_2_7_12_1","unstructured":"EMA Guideline on the clinical investigation of human plasma derived von Willebrand factor products (CPMP\/BPWG\/220\/02) available at:http:\/\/www.ema.europa.eu\/docs\/en_GB\/document_library\/Scientific_guideline\/2010\/01\/WC500067126.pdf.Accessed July 10 2012."},{"issue":"2","key":"e_1_2_7_13_1","article-title":"Interim analysis of a prospective observational study on Italian VWD patient, monitoring efficacy and safety during a formulation switch of a pasteurized FVIII\/VWF concentrate","volume":"7","author":"Morfini M","year":"2009","journal-title":"J Thromb Haemost"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1612977"},{"key":"e_1_2_7_15_1","first-page":"1279","article-title":"Efficacy and safety of factor VIII \u2044 von Willebrand's factor concentrate (Haemate\u2010P) in preventing bleeding during surgery or invasive procedures in patients with von Willebrand disease","volume":"88","author":"Franchini M","year":"2003","journal-title":"Haematologica"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1351-8216.2003.00816.x"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1351-8216.2003.00809.x"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2007.02588.x"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02206.x"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02445.x"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH10-10-0631"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH10-09-0616"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2010.10.005"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.mbc.0000167659.23262.18"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01851.x"},{"key":"e_1_2_7_26_1","doi-asserted-by":"crossref","first-page":"507a","DOI":"10.1182\/blood.V106.11.1782.1782","article-title":"Secondary long\u2010term prophylaxis in severe patients with von Willebrand's disease: an Italian cohort study","volume":"106","author":"Federici AB","year":"2005","journal-title":"Blood"},{"issue":"2","key":"e_1_2_7_27_1","first-page":"s26","article-title":"Prophylaxis of bleeding episodes in patients with von Willebrand's disease","volume":"6","author":"Federici AB","year":"2008","journal-title":"Blood Transfus"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2006.01.021"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2012.02752.x"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2007.01625.x"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2010.03864.x"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.12005","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.12005","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,14]],"date-time":"2023-10-14T22:14:00Z","timestamp":1697321640000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.12005"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,9,7]]},"references-count":30,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2013,1]]}},"alternative-id":["10.1111\/hae.12005"],"URL":"https:\/\/doi.org\/10.1111\/hae.12005","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,9,7]]}}}