{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T16:23:14Z","timestamp":1789402994023,"version":"build-2803163510"},"reference-count":40,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,5,30]],"date-time":"2025-05-30T00:00:00Z","timestamp":1748563200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,5,30]],"date-time":"2025-05-30T00:00:00Z","timestamp":1748563200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Patients with severe haemophilia A (HA) with no family history of haemophilia will be diagnosed upon their first bleeding event.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Herein, we studied the effects of lack of family history in HA and the subsequent delay of diagnosis on bleeding pattern and early treatment, as well as on the risk of inhibitor development. For this purpose, data on 1237 severe HA patients with known family history (\u201cpositive\u201d or \u201cnegative\u201d), born between 2000 and 2022, were collected in 29 participating centres.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>At diagnosis, 45.9% (554\/1208) of patients had a positive family history of HA and 54.1% (654\/1208) had a negative family history. A positive family history significantly shortened the time to diagnosis (8\u00a0months) and the treatment initiation (2\u00a0months). Prophylaxis was more frequently the first treatment in those with a positive family history compared to the negative family history group (21% vs. 13%). Bleeding was the main reason for first exposure day (ED) in both groups, but less frequently in the family history group than in those without a family history (67% vs. 80%). Positive family history was associated with fewer peak treatments at first five EDs (12% vs. 16%). In non\u2010inhibitor patients, bleeding occurred earlier in those with positive family history (9.2\u00a0months vs. 10.6\u00a0months). The inhibitor incidence was similar in both groups (33% vs. 30%), and a positive family history was associated with earlier inhibitor development (13\u00a0months vs. 15\u00a0months).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>The majority of patients presented without a family history of HA which led to a delayed diagnosis and treatment initiation.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hae.70018","type":"journal-article","created":{"date-parts":[[2025,5,30]],"date-time":"2025-05-30T06:38:33Z","timestamp":1748587113000},"page":"679-686","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Impact of Family History of Haemophilia on Diagnosis, Management and Outcomes in Severe Haemophilia"],"prefix":"10.1111","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-5248-8708","authenticated-orcid":false,"given":"Ana","family":"Mendoza","sequence":"first","affiliation":[{"name":"Hematology Department Hospital Universitario La Paz  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabel","family":"Rivas","sequence":"additional","affiliation":[{"name":"Hematology Department Hospital Universitario La Paz  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olga Ben\u00edtez","family":"Hidalgo","sequence":"additional","affiliation":[{"name":"Hematology Department Hospital Universitari Vall d'Hebron Experimental Hematology Vall d'Hebron Institute of Oncology (VHIO) Vall d'Hebron Barcelona Hospital Campus  Barcelona Spain"},{"name":"Medicine Department Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Rosa","family":"Cid","sequence":"additional","affiliation":[{"name":"Unidad de Hemostasia y Trombosis Hospital Universitario y Polit\u00e9cnico La Fe  Valencia Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6434-6244","authenticated-orcid":false,"given":"Martin","family":"Olivieri","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Pediatric Haemophilia Center Dr. von Hauner Children's Hospital LMU University Hospital Munich  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanna","family":"Ranta","sequence":"additional","affiliation":[{"name":"Pediatric Coagulation Unit Astrid Lindgren Children's Hospital Karolinska University Hospital  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5052-9274","authenticated-orcid":false,"given":"Veerle","family":"Labarque","sequence":"additional","affiliation":[{"name":"University Hospitals Leuven Service of Pediatric Haematology  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6058-8350","authenticated-orcid":false,"given":"Nadine G.","family":"Andersson","sequence":"additional","affiliation":[{"name":"Center for Thrombosis and Hemostasis Sk\u00e5ne University Hospital  Malm\u00f6 Sweden"},{"name":"Department of Pediatrics Sk\u00e5ne University Hospital  Lund Sweden"},{"name":"Department of Clinical Sciences and Pediatrics Lund University  Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3521-1863","authenticated-orcid":false,"given":"Marloes","family":"de 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