{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,30]],"date-time":"2026-06-30T08:38:36Z","timestamp":1782808716402,"version":"3.54.5"},"reference-count":15,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2024,5,13]],"date-time":"2024-05-13T00:00:00Z","timestamp":1715558400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2024,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>In the context of severe unexplained haemorrhage (SH), it is usual to seek haematological evaluation and investigate for an inherited rare bleeding disorder (IRBD). In such circumstances, appropriate screen can discriminate between IRBD and suspected child abuse. Yet, little information is available about the frequency of SH in the population of patients with IRBD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>To collect epidemiologic data about SH and IRBD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>The database of the FranceCoag network has collected information about IRBD since January 2004. Based on data gathered up to 16 March 2022, a retrospective search was conducted for of SH events having occurred before or at the time of IRBD diagnosis. Demographics and diagnosis circumstances were retrieved, as well as information about SH, defined as any life\u2010threatening bleeding or intracranial haemorrhage.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Among the 13,433 patients of the database, 109 (0.8%) fulfilled inclusion criteria including a known date of IRBD diagnosis, haemophilia A or B (HA\/HB) being the most frequent (82.5%). IRBD was discovered as a consequence of an SH event in 82.6% of the cases while CNS was involved in 55%. Severe and moderate HA\/HB and other severe IRBD presented significantly more intracranial haemorrhage (<jats:italic>p<\/jats:italic>\u00a0&lt;\u00a0.02) and a lower age at diagnosis (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.03).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These data support that any unusual SH should raise a suspicion of IRBD. Particularly before 1\u2010year of age, it is suggested to first confirm moderate or severe haemophilia and severe IRBD by standard coagulation tests (APTT, PT and fibrinogen), combined with a clotting FXIII assay as first\u2010line investigation. Subsequent assays of coagulation factors should be performed in the case of abnormal values, in second\u2010line investigation.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.15033","type":"journal-article","created":{"date-parts":[[2024,5,13]],"date-time":"2024-05-13T13:33:20Z","timestamp":1715607200000},"page":"981-987","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Severe haemorrhages leading to a diagnosis of rare bleeding disorder occur at a very young age: A study from the FranceCoag network"],"prefix":"10.1111","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6785-4332","authenticated-orcid":false,"given":"Sandrine","family":"Meunier","sequence":"first","affiliation":[{"name":"Hospices Civils de Lyon\u2010 Centre de R\u00e9f\u00e9rence H\u00e9mophilie \u2010 Unit\u00e9 d'H\u00e9mostase Clinique CHU de Lyon  Bron France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7368-4624","authenticated-orcid":false,"given":"Annie","family":"Harroche","sequence":"additional","affiliation":[{"name":"Service d'H\u00e9matologie Clinique Centre de Traitement de l'H\u00e9mophilie, H\u00f4pital Necker Enfants Malades, AP\u2010HP  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1182-4131","authenticated-orcid":false,"given":"Antoine","family":"Rauch","sequence":"additional","affiliation":[{"name":"Unit\u00e9 d'H\u00e9mostase Clinique et Biologique \u2013 P\u00f4le d'H\u00e9matologie Transfusion \u2010 Institut C\u0153ur Poumon  Lille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9cile","family":"Bally","sequence":"additional","affiliation":[{"name":"Service d'H\u00e9matologie Clinique Centre de Traitement de l'H\u00e9mophilie, H\u00f4pital Necker Enfants Malades, AP\u2010HP  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoann","family":"Huguenin","sequence":"additional","affiliation":[{"name":"Centre de Ressources et de Comp\u00e9tences Maladies H\u00e9morragiques Constitutionnelles Bordeaux France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romain","family":"Voltzenlogel","sequence":"additional","affiliation":[{"name":"FranceCoag Network, APHM  Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cl\u00e9mence","family":"Tab\u00e9l\u00e9","sequence":"additional","affiliation":[{"name":"FranceCoag Network, APHM  Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Herv\u00e9","family":"Chambost","sequence":"additional","affiliation":[{"name":"Centre de Traitement des H\u00e9mophiles H\u00f4pital Universitaire La Timone  Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martine","family":"Balen\u00e7on","sequence":"additional","affiliation":[{"name":"Service de P\u00e9diatrie CHU de Caen &amp; Universit\u00e9 de Caen Normandie  Caen France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"FranceCoag","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,5,13]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.18361"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2022-059277"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.transci.2018.10.009"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2013-304339"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2022-059276"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2012.04653.x"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.13941"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm11071969"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10654-018-0468-7"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.7326\/M19-1208"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.13823"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.16133"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.13461"},{"issue":"4","key":"e_1_2_9_15_1","first-page":"1","article-title":"Age at diagnosis of hemophilia: update from the French registry \u201cFranceCoag Network\u201d. 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Buenos Aires, Argentina.July 10\u201314, 2010","volume":"16","author":"Jousselme C","year":"2010","journal-title":"Haemophilia"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.2003.044073"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.15033","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,13]],"date-time":"2025-04-13T18:08:38Z","timestamp":1744567718000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.15033"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5,13]]},"references-count":15,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2024,7]]}},"alternative-id":["10.1111\/hae.15033"],"URL":"https:\/\/doi.org\/10.1111\/hae.15033","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,5,13]]},"assertion":[{"value":"2023-12-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-05-01","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-05-13","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}