{"id":1044,"date":"2020-03-19T14:39:28","date_gmt":"2020-03-19T13:39:28","guid":{"rendered":"https:\/\/www.francecoag.org\/?post_type=publications_science&#038;p=1044"},"modified":"2025-03-19T14:50:19","modified_gmt":"2025-03-19T13:50:19","slug":"bouttefroy-s-et-al-congenital-factor-xiii-deficiency-comprehensive-overview-of-the-francecoag-cohort","status":"publish","type":"publications_science","link":"https:\/\/www.francecoag.org\/en\/publications_science\/bouttefroy-s-et-al-congenital-factor-xiii-deficiency-comprehensive-overview-of-the-francecoag-cohort\/","title":{"rendered":"Bouttefroy S et al\u00a0: Congenital factor XIII deficiency: comprehensive overview of the FranceCoag cohort"},"content":{"rendered":"<p><a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/31414482\/\">https:\/\/pubmed.ncbi.nlm.nih.gov\/31414482\/<\/a><\/p>\n<header id=\"heading\" class=\"heading\">\n<div id=\"full-view-heading\" class=\"full-view\">\n<ul id=\"full-view-identifiers\" class=\"identifiers\">\n<li><span class=\"identifier pubmed\"> <span class=\"id-label\"> PMID: <\/span> <strong class=\"current-id\" title=\"PubMed ID\">31414482<\/strong> <\/span><\/li>\n<li><span class=\"identifier doi\"> <span class=\"id-label\"> DOI: <\/span> <a class=\"id-link\" href=\"https:\/\/doi.org\/10.1111\/bjh.16133\" target=\"_blank\" rel=\"noopener\" data-ga-category=\"full_text\" data-ga-action=\"DOI\"> 10.1111\/bjh.16133 <\/a> <\/span><\/li>\n<\/ul>\n<\/div>\n<\/header>\n<div id=\"abstract\" class=\"abstract\">\n<h2 class=\"title\">Abstract<\/h2>\n<div id=\"eng-abstract\" class=\"abstract-content selected\">\n<p>This FranceCoag network study assessed 33 patients with congenital factor XIII (FXIII) deficiency presenting FXIII levels &lt;10 iu\/dl. Diagnosis was based on abnormal bleeding in 29 patients, a positive family history in 2, recurrent miscarriages in 1 and was fortuitous in 1. Eighteen patients (62\u00b71%) presented life-threatening umbilical or intracranial haemorrhages (ICH). Seven of the 15 patients who experienced ICH were diagnosed but untreated, including 3 with secondary neurological sequelae. All pregnancies without prophylaxis (26\/26) led to miscarriages versus 3\/16 with prophylaxis. In patients exhibiting FXIII levels &lt;10 iu\/dl, prophylaxis could be discussed at diagnosis and at pregnancy. Further controlled prospective studies are needed.<\/p>\n<\/div>\n<p><strong class=\"sub-title\"> Keywords: <\/strong> epidemiology; factor XIII; prophylaxis; rare bleeding disorder; registry.<\/p>\n<\/div>\n<p id=\"copyright\" class=\"copyright\">\u00a9 2019 British Society for Haematology and John Wiley &amp; Sons Ltd.<\/p>\n","protected":false},"parent":0,"template":"","keyword":[],"class_list":["post-1044","publications_science","type-publications_science","status-publish","hentry"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.5 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Bouttefroy S et al\u00a0: Congenital factor XIII deficiency: comprehensive overview of the FranceCoag cohort - FranceCoag<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.francecoag.org\/en\/publications_science\/bouttefroy-s-et-al-congenital-factor-xiii-deficiency-comprehensive-overview-of-the-francecoag-cohort\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Bouttefroy S et al\u00a0: Congenital factor XIII deficiency: comprehensive overview of the FranceCoag cohort - 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