{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,6]],"date-time":"2026-11-06T11:39:46Z","timestamp":1793965186571,"version":"4.8.0"},"reference-count":27,"publisher":"Georg Thieme Verlag KG","issue":"04","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Thromb Haemost"],"published-print":{"date-parts":[[2011]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>In patients with von Willebrand disease (VWD) replacement therapy with factor VIII\/von Willebrand (VWF) concentrates is increasingly applied as prophylactic regimen. Since 2000, 82 consecutively enrolled patients with clinically relevant bleeding episodes (spontaneous, peri- or postoperative) were diagnosed with VWD [type 1: 42\/82; type 2: 24\/82; type 3: 13\/82; acquired: 3\/82]. In all patients, decision for initiating prophylaxis was based on a bleeding score &gt; 2 prior to diagnosis, concomitant with recurrent bleeds associated with anaemia in patients with on-demand VWD therapy. We report results on secondary prophylactic VWF replacement therapy applied in 32 patients [children n=13; adolescents n=7; adults n=12] with VWD [type 1: 4; type 2: 15; type 3: 13], 15 of which were females, and nine of these at the reproductive period. Eight patients were treated with Humate P\u2192 or Wilate\u2192 (n=24). Median [min-max] dose [vWF:RCo] was 40 [20\u201347] IU\/kg, 23 patients were given substitution therapy twice weekly, seven patients three times a week, and two children four times per week. Within a 12-month-period haemoglobin concentrations returned to normal values. Median duration of prophylaxis was three years. Recurrent bleeding episodes stopped in 31 of 32 patients, whereas inhibitors developed in one. Following a 12-month observation period the monthly bleeding frequency and the bleeding score was significantly reduced [3 vs. 0.07; 3 vs. 0: p&lt; 0.001], compared to the preprophylaxis\/pre-diagnostic values. The use of secondary prophylactic VWF replacement therapy is an effective tolerated treatment modality, highly beneficial for patients with VWD, who present with recurrent bleeding events during on-demand therapy.<\/jats:p>","DOI":"10.1160\/th10-09-0616","type":"journal-article","created":{"date-parts":[[2011,2,8]],"date-time":"2011-02-08T12:49:41Z","timestamp":1297169381000},"page":"597-604","source":"Crossref","is-referenced-by-count":32,"title":["Long-term secondary prophylaxis in children, adolescents and young adults with von Willebrand disease"],"prefix":"10.1160","volume":"105","author":[{"given":"Susan","family":"Halimeh","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"Kr\u00fcmpel","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hannelore","family":"Rott","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadja","family":"Bogdanova","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrich","family":"Budde","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Manner","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Britta","family":"Faeser","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rolf","family":"Mesters","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrike","family":"Nowak-G\u00f6ttl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2017,11,28]]},"reference":[{"key":"10.1160\/TH10-09-0616-1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01713.x"},{"key":"10.1160\/TH10-09-0616-2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-09-2892"},{"key":"10.1160\/TH10-09-0616-3","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198005083021902"},{"key":"10.1160\/TH10-09-0616-4","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2000.02507.x"},{"issue":"Suppl 2","key":"10.1160\/TH10-09-0616-5","first-page":"s26","volume":"6","author":"Federici","year":"2008","journal-title":"Blood Transfus"},{"key":"10.1160\/TH10-09-0616-6","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2006.01.022"},{"key":"10.1160\/TH10-09-0616-7","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.11124"},{"key":"10.1160\/TH10-09-0616-8","doi-asserted-by":"publisher","DOI":"10.1055\/s-2006-949665"},{"key":"10.1160\/TH10-09-0616-9","doi-asserted-by":"publisher","DOI":"10.1055\/s-2006-949667"},{"key":"10.1160\/TH10-09-0616-10","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2006.01.021"},{"key":"10.1160\/TH10-09-0616-11","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01851.x"},{"key":"10.1160\/TH10-09-0616-12","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(09)70153-7"},{"key":"10.1160\/TH10-09-0616-13","first-page":"117","volume":"7","author":"Mannucci","year":"2009","journal-title":"Blood Transfus"},{"key":"10.1160\/TH10-09-0616-14","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1055\/s-0037-1613022","volume":"87","author":"Lillicrap","year":"2002","journal-title":"Thromb Haemost"},{"key":"10.1160\/TH10-09-0616-15","first-page":"1279","volume":"88","author":"Franchini","year":"2003","journal-title":"Haematologica"},{"key":"10.1160\/TH10-09-0616-16","doi-asserted-by":"publisher","DOI":"10.1046\/j.1351-8216.2003.00809.x"},{"key":"10.1160\/TH10-09-0616-17","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2007.02588.x"},{"key":"10.1160\/TH10-09-0616-18","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2006.01.014"},{"key":"10.1160\/TH10-09-0616-19","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2005.01663.x"},{"key":"10.1160\/TH10-09-0616-20","doi-asserted-by":"publisher","DOI":"10.1177\/1076029606293422"},{"key":"10.1160\/TH10-09-0616-21","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2007.01643.x"},{"key":"10.1160\/TH10-09-0616-22","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01813.x"},{"key":"10.1160\/TH10-09-0616-23","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2000.00383.x"},{"key":"10.1160\/TH10-09-0616-24","doi-asserted-by":"crossref","unstructured":"James AH, Kouides PA, Kadir R, et al. Von Willebrand disease and other bleeding disorders in women: consensus on diagnosis and management from an international expert panel. 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