{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T07:00:23Z","timestamp":1791961223402,"version":"4.3.4"},"reference-count":25,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2022,11,24]],"date-time":"2022-11-24T00:00:00Z","timestamp":1669248000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2023,1]]},"abstract":"Abstract<\/jats:title>Introduction<\/jats:title>Factor IX replacement therapy is used for treatment and prophylaxis of bleeding in haemophilia B. rIX\u2010FP is an extended half\u2010life albumin\u2010fusion protein, which, in clinical studies, has demonstrated prolonged dosing intervals up to 21 days for routine prophylaxis, providing therapeutic benefit.<\/jats:p><\/jats:sec>Aims<\/jats:title>To describe dosing frequency and consumption (primary endpoint), efficacy and safety of rIX\u2010FP treatment during routine clinical practice in Italy.<\/jats:p><\/jats:sec>Methods<\/jats:title>Patients with moderate\/severe haemophilia B on prophylaxis with rIX\u2010FP for \u22656 months, were enrolled in this observational study from October 2017 to February 2019 and followed\u2010up for 2 years. Descriptive analysis included prospective and retrospective data (12 months prior to switching to rIX\u2010FP).<\/jats:p><\/jats:sec>Results<\/jats:title>Data were collected from 59 male patients (median age 30.1 years) enrolled by 23 Italian centres. Of them, 50 were on prophylaxis during the entire observation period and completed the study. The infusion frequency changed from 2\u20133 times\/week in 86.0% of patients with previous treatment, to less than once a week in 84.0% of patients treated with rIX\u2010FP at the 2nd\u2010year follow\u2010up. The annual number of infusions decreased by about 70%, whereas the mean FIX activity trough level increased from 3.8% to 14.4% (mean\u00a0>\u00a010% in all the infusion regimens). Median Annualised Bleeding Rate of .0 was achieved across all prophylaxis regimens. Subjects with zero bleedings increased from 66.0% to 78.0% with rIX\u2010FP.<\/jats:p><\/jats:sec>Conclusion<\/jats:title>Treatment with rIX\u2010FP reduced infusion frequency, while providing higher FIX trough levels with substantial benefit in terms of annualised bleeding rate and a good safety profile.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14689","type":"journal-article","created":{"date-parts":[[2022,11,24]],"date-time":"2022-11-24T22:28:13Z","timestamp":1669328893000},"page":"135-144","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["IDEAL study: A real\u2010world assessment of pattern of use and clinical outcomes with recombinant coagulation factor IX albumin fusion protein (rIX\u2010FP) in patients with haemophilia B in Italy"],"prefix":"10.1111","volume":"29","author":[{"given":"Annarita","family":"Tagliaferri","sequence":"first","affiliation":[{"name":"Regional Reference Centre for Inherited Bleeding Disorders University Hospital of Parma Parma Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8078-8402","authenticated-orcid":false,"given":"Angelo Claudio","family":"Molinari","sequence":"additional","affiliation":[{"name":"Regional Reference Centre for Haemorrhagic Diseases IRCCS Istituto Giannina Gaslini Genova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Flora","family":"Peyvandi","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology and Transplantation Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico Haemophilia and Thrombosis Centre Universit\u00e0 degli Studi di Milano Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3697-706X","authenticated-orcid":false,"given":"Antonio","family":"Coppola","sequence":"additional","affiliation":[{"name":"Regional Reference Centre for Inherited Bleeding Disorders University Hospital of Parma Parma Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Demartis","sequence":"additional","affiliation":[{"name":"Department of Oncology Centre for Bleeding Disorders and Coagulation Careggi University Hospital Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Biasoli","sequence":"additional","affiliation":[{"name":"Haemophilia and Transfusion Centre \u201cBufalini\u201d Hospital Cesena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Borchiellini","sequence":"additional","affiliation":[{"name":"Regional Reference Centre for Bleeding and Thrombotic Disorders \u201cCitt\u00e0 della Salute e della Scienza\u201d University Hospital Turin Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorina","family":"Cultrera","sequence":"additional","affiliation":[{"name":"Haemophilia Regional Reference Centre Haematology Unit Policlinico \"G. Rodolico \u2010 S. Marco\u201d Hospital Catania Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raimondo","family":"De Cristofaro","sequence":"additional","affiliation":[{"name":"Haemorrhagic and Thrombotic Disease Service Area of Haematological and Oncological Sciences IRCCS Fondazione Policlinico Universitario \u201cA. Gemelli\u201d Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filomena","family":"Daniele","sequence":"additional","affiliation":[{"name":"Haemostasis and Thrombosis Service Ospedale Civile dell'Annunziata Cosenza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Giordano","sequence":"additional","affiliation":[{"name":"\u201cB. Trambusti\u201d General and Specialised Paediatrics Unit \u201cGiovanni XXIII\u201d Hospital University of Bari Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuela","family":"Marchesini","sequence":"additional","affiliation":[{"name":"Haemophilia Centre, Internal and Cardiovascular Medicine \u201cSanta Maria della Misericordia\u201d University Hospital Perugia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurizio","family":"Margaglione","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine University of Foggia Foggia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renato","family":"Marino","sequence":"additional","affiliation":[{"name":"Haemophilia and Thrombosis Centre University Hospital Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Berardino","family":"Pollio","sequence":"additional","affiliation":[{"name":"Regional Reference Centre for Inherited Bleeding and Thrombotic Disorders Transfusion Medicine \u201cRegina Margherita\u201d Children Hospital Turin Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Radossi","sequence":"additional","affiliation":[{"name":"Onco\u2010Haematology Unit Istituto Oncologico Veneto Castelfranco Veneto Hospital Castelfranco Veneto Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7181-447X","authenticated-orcid":false,"given":"Cristina","family":"Santoro","sequence":"additional","affiliation":[{"name":"Department of Haematology University Hospital Policlinico Umberto I Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5081-8201","authenticated-orcid":false,"given":"Rita Carlotta","family":"Santoro","sequence":"additional","affiliation":[{"name":"Regional Reference Centre for Haemophilia and Coagulation Diseases Azienda Ospedaliera Pugliese\u2010Ciaccio Catanzaro Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sergio","family":"Siragusa","sequence":"additional","affiliation":[{"name":"Haematology Unit Thrombosis and Haemostasis Reference Regional Centre University of Palermo Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8833-1514","authenticated-orcid":false,"given":"Gianluca","family":"Sottilotta","sequence":"additional","affiliation":[{"name":"Haemophilia Centre Grande Ospedale Metropolitano Reggio Calabria Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Tosetto","sequence":"additional","affiliation":[{"name":"Haematology Department Haemostasis and Thrombosis Unit San Bortolo Hospital Vicenza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lydia","family":"Piscitelli","sequence":"additional","affiliation":[{"name":"SSD Congenital Haemorrhagic Diseases IRCCS Azienda Ospedaliero\u2010Universitaria di Bologna Bologna Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Rosaria","family":"Villa","sequence":"additional","affiliation":[{"name":"Haemophilia and Thrombosis Centre Haematology Unit \u201cOspedale del Mare\u201d Hospital Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ezio","family":"Zanon","sequence":"additional","affiliation":[{"name":"Department of Medicine Haemophilia Centre University Hospital of Padua Padua Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adele","family":"Finardi","sequence":"additional","affiliation":[{"name":"CSL Behring Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene","family":"Schiavetti","sequence":"additional","affiliation":[{"name":"Hippocrates Research Genova Italy"},{"name":"Department of Health Sciences University of Genoa Genoa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniella","family":"Vaccari","sequence":"additional","affiliation":[{"name":"Hippocrates Research Genova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4973-1317","authenticated-orcid":false,"given":"Giancarlo","family":"Castaman","sequence":"additional","affiliation":[{"name":"Department of Oncology Centre for Bleeding Disorders and Coagulation Careggi University Hospital Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,11,24]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14046"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2012.02835.x"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-01-528414"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2019.242735"},{"issue":"4","key":"e_1_2_8_6_1","first-page":"634","article-title":"Genetic fusion to albumin improves the pharmacokinetic properties of factor IX","volume":"102","author":"Metzner HJ","year":"2009","journal-title":"Thromb Haemost"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2003.00840.x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2007.06703.x"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-05-429688"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(13)70150-6"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.13735"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood\u20102015\u201009\u2010669234"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.14778"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12325\u2010020\u201001300\u20106"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1080\/03007995.2019.1662675"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14018"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm11041071"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1080\/17474086.2018.1489719"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.2147\/PPA.S92520"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.2147\/PPA.S139851"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12959"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa067659"},{"key":"e_1_2_8_23_1","article-title":"Clotting factor concentrates given to prevent bleeding and bleeding\u2010related complications in people with hemophilia A or B","volume":"9","author":"Iorio A","year":"2011","journal-title":"Cochrane Database Syst Rev"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.13985"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02383.x"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14152"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.14689","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/hae.14689","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.14689","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,17]],"date-time":"2023-08-17T22:16:30Z","timestamp":1692310590000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.14689"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11,24]]},"references-count":25,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2023,1]]}},"alternative-id":["10.1111\/hae.14689"],"URL":"https:\/\/doi.org\/10.1111\/hae.14689","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,11,24]]},"assertion":[{"value":"2022-06-17","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-10-22","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-11-24","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}