{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T11:01:10Z","timestamp":1772794870261,"version":"3.50.1"},"reference-count":31,"publisher":"Wiley","issue":"s76","license":[{"start":{"date-parts":[[2014,6,24]],"date-time":"2014-06-24T00:00:00Z","timestamp":1403568000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2014,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Haemophilia A is a sex\u2010linked disorder characterised chiefly by recurrent, spontaneous joint and muscle bleedings resulting from deficiency of factor <jats:styled-content style=\"fixed-case\">VIII<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content>). Recurrent joint bleeds result in haemophilic arthropathy. Unless treated with factor replacement therapy, many patients with severe haemophilia become disabled. The first clinical evidence favouring prophylaxis originated from the studies in <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>weden and the <jats:styled-content style=\"fixed-case\">N<\/jats:styled-content>etherlands in the 1960s. Later on, it was shown that prophylaxis could prevent arthropathy, if started early in life, or slow its progression in adults with established arthropathy. The optimal dosing of <jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content> in long\u2010term prophylaxis has still not been determined, and there is growing evidence that the dose and frequency of <jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content> should be individualised. We conducted a systematic search of <jats:styled-content style=\"fixed-case\">P<\/jats:styled-content>ub<jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>ed to identify all relevant articles on <jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content> prophylaxis in severe haemophilia <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>. We focused on articles with detailed information about individualisation of prophylaxis. Long\u2010term prophylaxis in haemophilia was introduced in <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>weden in the late 1950s. However, standard prophylactic regimens may not be appropriate for all patients with severe haemophilia. Factors such as age, joint status, co\u2010morbidities and differences in pharmacokinetics lead to interindividual variation in factor requirement. Dose tailoring of <jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content> by clinical outcome was first described in 1994. Since then, several dose\u2010finding studies questioned the necessity to maintain preinfusion levels of <jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content> above 1%. Individualising prophylaxis by dose tailoring is now recommended. Each country should adopt policies for individualising prophylaxis in patients with severe haemophilia. This would lead to a better distribution of the available source of factor concentrates.<\/jats:p>","DOI":"10.1111\/ejh.12370","type":"journal-article","created":{"date-parts":[[2014,6,24]],"date-time":"2014-06-24T09:53:54Z","timestamp":1403603634000},"page":"16-20","source":"Crossref","is-referenced-by-count":28,"title":["Methods for individualising factor <scp>VIII<\/scp> dosing in prophylaxis"],"prefix":"10.1111","volume":"93","author":[{"given":"Muhlis Cem","family":"Ar","sequence":"first","affiliation":[{"name":"Department of Haematology Istanbul University Cerrahpasa Medical Faculty Istanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ines","family":"Vaide","sequence":"additional","affiliation":[{"name":"Department of Haematology North Estonian Medical Centre Tallinn Estonia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erik","family":"Berntorp","sequence":"additional","affiliation":[{"name":"Lund University Centre for Thrombosis and Haemostasis Sk\u00e5ne University Hospital Malm\u00f6 Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sven","family":"Bj\u00f6rkman","sequence":"additional","affiliation":[{"name":"Department of Pharmaceutical Biosciences Uppsala University Uppsala Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,6,24]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.1.6161.469"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2796.1994.tb00815.x"},{"issue":"1","key":"e_1_2_5_4_1","first-page":"1","article-title":"Haemophilia in Sweden VII. 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