{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,15]],"date-time":"2026-05-15T03:07:37Z","timestamp":1778814457442,"version":"3.51.4"},"reference-count":17,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2013,4,22]],"date-time":"2013-04-22T00:00:00Z","timestamp":1366588800000},"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":[[2013,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>The prevalence of inhibitors in haemophilia B is significantly lower than that of patients with haemophilia A. However, the peculiar occurrence of allergic reactions associated with the onset of inhibitor in haemophilia B (HB) may render immune tolerance a risky procedure. We have carried out a detailed survey among all the Italian Hemophilia Centers to analyse all the patients with HB and inhibitors. A total of eight patients were reported among 282 living patients (2.8%) with severe factor IX (FIX) deficiency (FIX\u00a0&lt;\u00a01\u00a0U\u00a0dL<jats:sup>\u22121<\/jats:sup>). In addition, two deceased patients were also identified. Six patients carried nonsense mutations while in four partial or complete gene deletions were detected. Three patients (one deceased) had history of allergic\/anaphylactic reaction upon substitutive treatment, which in one case was recurrent and resolved after switching to plasma derived FIX. Immune tolerance was adopted in five patients and in four complete response was achieved while in the remaining it was partial. No nephrotic syndrome was observed. Our data confirm that inhibitors in HB occur in patients with null mutations or complete\/partial gene deletion. Immune tolerance can be achieved also in HB patients, without allergic reactions or nephrotic syndrome upon replacement therapy.<\/jats:p>","DOI":"10.1111\/hae.12158","type":"journal-article","created":{"date-parts":[[2013,4,22]],"date-time":"2013-04-22T04:12:38Z","timestamp":1366603958000},"page":"686-690","source":"Crossref","is-referenced-by-count":27,"title":["Inhibitors in haemophilia B: the Italian experience"],"prefix":"10.1111","volume":"19","author":[{"given":"G.","family":"Castaman","sequence":"first","affiliation":[{"name":"Department of Cell Therapy and Hematology Hemophilia and Thrombosis Center San Bortolo Hospital Vicenza"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Bonetti","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology and Hematology University of Verona Verona"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Messina","sequence":"additional","affiliation":[{"name":"Hemophilia Center University of Torino Torino"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Morfini","sequence":"additional","affiliation":[{"name":"Hemophilia Center University Florence Florence"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Rocino","sequence":"additional","affiliation":[{"name":"Hemophilia Center San G. Bosco Hospital Naples"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F. A.","family":"Scaraggi","sequence":"additional","affiliation":[{"name":"Hemophilia Center University of Bari Bari"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Tagariello","sequence":"additional","affiliation":[{"name":"Department of Hematology Transfusion and Hemophilia Center Castelfranco Veneto Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"Italian Association of Hemophilia Centers","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2013,4,22]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1517\/14656566.6.9.1517"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(92)90874-3"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0038-1648507"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.1996.tb00006.x"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-199701000-00003"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V89.3.1115"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02039.x"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01679.x"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2007.01497.x"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2005.01161.x"},{"key":"e_1_2_7_12_1","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1182\/blood.V66.3.728.728","article-title":"Hemophilia B with inhibitor: molecular analysis of the subtotal deletion of the factor IX gene","volume":"66","author":"Hassan HJ","year":"1985","journal-title":"Blood"},{"key":"e_1_2_7_13_1","first-page":"635","article-title":"Molecular genotyping of the Italian cohort of patients with hemophilia B","volume":"90","author":"Belvini D","year":"2005","journal-title":"Haematologica"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01361.x"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01880.x"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2007.06657.x"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/9780470987124.ch17"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.1999.t01-1-00303.x"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.12158","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.12158","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T09:08:18Z","timestamp":1696324098000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.12158"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,4,22]]},"references-count":17,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2013,9]]}},"alternative-id":["10.1111\/hae.12158"],"URL":"https:\/\/doi.org\/10.1111\/hae.12158","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,4,22]]}}}