{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T15:00:55Z","timestamp":1787497255671,"version":"build-2736575974"},"reference-count":71,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2021,5,22]],"date-time":"2021-05-22T00:00:00Z","timestamp":1621641600000},"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":[[2021,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Vitamin K\u2013dependent factor X (FX) plays an important role in thrombin formation, and a deficiency in FX can cause impaired coagulation, the severity of which is usually correlated with the degree of deficiency. Due to the critical role that FX plays in the coagulation cascade, FX deficiency is associated with a higher risk of bleeding than deficiencies in other coagulation factors. Patients with the hereditary autosomal\u2010recessive homozygous form of FX deficiency, which occurs in approximately 1:1,000,000 individuals worldwide, are often diagnosed when they present with spontaneous life\u2010threatening haemorrhage (most often intracranial haemorrhage) during the first month of life. In addition to central nervous system bleeds, other severe bleeding types experienced by such patients may include umbilical cord bleeding, gastrointestinal or pulmonary haemorrhage, intramuscular haematomas and\/or haemarthrosis. Delayed treatment or inadequate replacement of FX may result in developmental delays, musculoskeletal disabilities or death. The high risk of recurrent severe bleeding necessitates prophylactic replacement therapy for many individuals with severe FX deficiency. Available products for replacement therapy include plasma\u2010derived FX concentrate and prothrombin complex concentrates. Fresh\u2010frozen plasma may be used when concentrates are not available but is a less efficient means of FX replacement. This article reviews the literature on severe bleeding in individuals with hereditary FX deficiency and discusses current treatment options.<\/jats:p>","DOI":"10.1111\/hae.14223","type":"journal-article","created":{"date-parts":[[2021,5,22]],"date-time":"2021-05-22T07:19:50Z","timestamp":1621667990000},"page":"531-543","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Occurrence and management of severe bleeding episodes in patients with hereditary factor X deficiency"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0069-8176","authenticated-orcid":false,"given":"Michael D.","family":"Tarantino","sequence":"first","affiliation":[{"name":"Bleeding and Clotting Disorders Institute University of Illinois College of Medicine Peoria IL USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,5,22]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01856.x"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1998.00806.x"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13058"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2002.00633.x"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation-2016.1.663"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01814.x"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12368"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/MBC.0000000000000204"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2012.04653.x"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V70.1.165.165"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2012.04844.x"},{"key":"e_1_2_9_13_1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.33309\/2639-8354.010101","article-title":"Mutations seen in families with factor X deficiency composed of only heterozygous patients who present a bleeding tendency","volume":"1","author":"Girolami A","year":"2018","journal-title":"Clin Res Hematol"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.13729"},{"key":"e_1_2_9_15_1","first-page":"389","article-title":"Treatment of a subdural hematoma and long\u2010term secondary prophylaxis in a patient with severe factor X (FX) deficiency treated with a high\u2010purity plasma\u2010derived factor X (pdFX) concentrate. 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