{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,8]],"date-time":"2026-02-08T03:11:40Z","timestamp":1770520300840,"version":"3.49.0"},"reference-count":38,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2012,11,20]],"date-time":"2012-11-20T00:00:00Z","timestamp":1353369600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2013,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Recurrent bleeding into joints initiates a sequence of events leading to a progressive joint damage in people with severe haemophilia. This is a continuous process during childhood and adolescence, therefore joint abnormalities may be minimal on physical examination in very young children \u2013 even those receiving on\u2010demand treatment. The aim of our study was to quantify the burden of arthropathy in Lithuanian patients who had been treated exclusively by on\u2010demand substitution and compare their physical joint health with age\u2010matched Danish patients who received prophylaxis from an early age. Boys, aged 4\u201317\u00a0years, with severe haemophilia and no signs of inhibitors were included in the study. Joint outcome based on the Haemophilia Joint Health Score (<jats:styled-content style=\"fixed-case\">HJHS<\/jats:styled-content>) was analysed in two different treatment groups and compared within the matched pairs. In total, 32 (16 in each treatment group) patients were enroled. A total of 192 joints were evaluated. Joint status according to treatment strategy was strikingly different: 27.4 for on\u2010demand vs. 3.3 for prophylaxis (&lt;0.001) group. Significance of the difference in joint status comparing different treatment strategies was equally strong both in younger (4\u20139\u00a0years) and older (10\u201317\u00a0years) patient groups: 2.2 vs. 12.5 (<jats:italic>P<\/jats:italic>\u00a0= 0.0002) and 3.9 vs. 36.3 (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.0001) respectively. The results further demonstrate the unequivocal effect of prophylaxis on joint status and give an insight into early and late manifestations of joint impairment based on the <jats:styled-content style=\"fixed-case\">HJHS<\/jats:styled-content> in haemophilia patients with treatment on\u2010demand compared with joint changes that may develop over the time with the preventative treatment.<\/jats:p>","DOI":"10.1111\/hae.12058","type":"journal-article","created":{"date-parts":[[2012,11,20]],"date-time":"2012-11-20T15:46:26Z","timestamp":1353426386000},"page":"212-218","source":"Crossref","is-referenced-by-count":22,"title":["Progression of haemophilic arthropathy in children: a Lithuanian \u2013 Danish comparative study"],"prefix":"10.1111","volume":"19","author":[{"given":"S.","family":"Saulyte Trakymiene","sequence":"first","affiliation":[{"name":"Clinic of Children's Diseases, Vilnius University and Centre for Pediatric Hematology\/Oncology Children's Hospital, Vilnius University Hospital Santariskiu Klinikos Vilnius Lithuania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N.","family":"Clausen","sequence":"additional","affiliation":[{"name":"Division of Haematology Department of Paediatrics Aarhus University Hospital Skejby Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L. H.","family":"Poulsen","sequence":"additional","affiliation":[{"name":"Department of Biochemistry Centre for Haemophilia and Thrombosis Aarhus University Hospital Skejby Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Ingerslev","sequence":"additional","affiliation":[{"name":"Department of Biochemistry Centre for Haemophilia and Thrombosis Aarhus University Hospital Skejby Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Rageliene","sequence":"additional","affiliation":[{"name":"Clinic of Children's Diseases, Vilnius University and Centre for Pediatric Hematology\/Oncology Children's Hospital, Vilnius University Hospital Santariskiu Klinikos Vilnius Lithuania"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2012,11,20]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02450.x"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1996.420960.x"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1976.tb00973.x"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1980.tb07341.x"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2007.01532.x"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/00008480-200202000-00008"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01281.x"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2796.1997.130135000.x"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2004.00998.x"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.01953.x"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2002.00694.x"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2002.00695.x"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V99.7.2337"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1351-8216.2001.00120.x"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa067659"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830470209"},{"issue":"3","key":"e_1_2_8_18_1","first-page":"12","article-title":"Experience with prophylaxis in Germany","volume":"30","author":"Schramm W","year":"1993","journal-title":"Semin Hematol"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2796.1994.tb00815.x"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.02580.x"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1159\/000208615"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2001.00545.x"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2796.1992.tb00546.x"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2011.04214.x"},{"key":"e_1_2_8_25_1","first-page":"CD003429","article-title":"Clotting factor concentrates given to prevent bleeding and bleeding\u2010related complications in people with hemophilia A or B","volume":"9","author":"Iorio A","year":"2011","journal-title":"Cochrane Database Syst Rev"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2010.029868"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01268.x"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/PL00003616"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2005.01061.x"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2011.02513.x"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2011.02615.x"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02178.x"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01750.x"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.20353"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01312.x"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02385.x"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0609.2012.01750.x"},{"issue":"4","key":"e_1_2_8_38_1","first-page":"113","article-title":"Can the Haemophilia Joint Health Score (HJHS) identify differences in joint status in boys with severe haemophilia receiving prophylaxis?","volume":"16","author":"Bladen M","year":"2010","journal-title":"Haemophilia (Abstracts of the XXIXth International Congress of the World Federation of Hemophilia)"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.20353"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.12058","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.12058","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,30]],"date-time":"2023-10-30T06:23:25Z","timestamp":1698647005000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.12058"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,11,20]]},"references-count":38,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2013,3]]}},"alternative-id":["10.1111\/hae.12058"],"URL":"https:\/\/doi.org\/10.1111\/hae.12058","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,11,20]]}}}