{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,8,6]],"date-time":"2024-08-06T10:28:12Z","timestamp":1722940092372},"reference-count":29,"publisher":"Georg Thieme Verlag KG","issue":"08","funder":[{"name":"unrestricted grant from CLS Behring and the Dutch Hemophilia Foundation."}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Thromb Haemost"],"published-print":{"date-parts":[[2017]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Assessment of clinical outcome after joint bleeding is essential to identify joint damage and optimise treatment, to prevent disability. However, disease-specific tools to assess the musculoskeletal status in patients with von Willebrand disease (VWD) are lacking. We aimed to determine validity and reliability of the Haemophilia Joint Health Score (HJHS) and Haemophilia Activities List (HAL) in patients with Von Willebrand disease (VWD). Ninety-six patients with VWD were included (mean age 46 years) of whom 27 had more than five documented joint bleeds. The HJHS was performed in all patients and all patients completed the HAL and Impact on Participation and Autonomy (IPA) questionnaires. Health-related quality of life (SF36) results were obtained from the prior \u2018Willebrand in the Netherlands\u2019 study. Joint X-rays of knees, elbows and ankles were scored according to Pettersson (PS). Internal consistency of the HJHS (Cronbach\u2019s \u03b1 (\u03b1)=0.75) and HAL (\u03b1=0.89) were good. Inter-observer agreement of the HJHS was good (ICC 0.84; Limits of Agreement \u00b1 10.3). The HJHS showed acceptable correlation with the X-ray PS (Spearman\u2019s r (rs)&gt;0.60 all joints) and HAL (rs=0.71). The HAL also showed acceptable correlation with the SF36 physical functioning (rs=0.65) and IPA (rs=0.69). Hypothesis testing showed adequate discriminative power of both instruments: in patients with a history of &gt;5 versus \u2264 5 joint bleeds (median HJHS 10 vs 2 (p&lt;0.01); median HAL 77 vs 98 (p&lt;0.01)), independent from age. In conclusion, both the HJHS and HAL are feasible to assess clinical outcome after joint bleeds in VWD.<\/jats:p>","DOI":"10.1160\/th16-12-0967","type":"journal-article","created":{"date-parts":[[2017,5,11]],"date-time":"2017-05-11T09:52:12Z","timestamp":1494496332000},"page":"1465-1470","source":"Crossref","is-referenced-by-count":5,"title":["Joint assessment in von Willebrand disease"],"prefix":"10.1160","volume":"117","author":[{"given":"Karin","family":"van Galen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Merel","family":"Timmer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Piet","family":"de Kleijn","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kathelijn","family":"Fischer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wouter","family":"Foppen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roger","family":"Schutgens","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeroen","family":"Eikenboom","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karina","family":"Meijer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marjon","family":"Cnossen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karin","family":"Fijnvandraat","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johanna","family":"van der Bom","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Britta","family":"Larosvan Gorkom","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Frank","family":"Leebeek","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eveline","family":"Mauser-Bunschoten","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"on behalf of the Win study group","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2017,11,22]]},"reference":[{"key":"10.1160\/TH16-12-0967-1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1601561"},{"key":"10.1160\/TH16-12-0967-2","doi-asserted-by":"crossref","unstructured":"Ahlberg A. Haemophilia in Sweden. VII. Incidence, treatment and prophylaxis of arthropathy and other musculo-skeletal manifestations of haemophilia A and B. Acta Orthop Scand Suppl 1965; Suppl 77: 3-132","DOI":"10.3109\/ort.1965.36.suppl-77.01"},{"key":"10.1160\/TH16-12-0967-3","doi-asserted-by":"publisher","DOI":"10.3109\/17453677008991542"},{"key":"10.1160\/TH16-12-0967-4","first-page":"1","volume":"238","author":"Silwer","year":"1973","journal-title":"Acta Paediatr Scand Suppl"},{"key":"10.1160\/TH16-12-0967-5","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0455.2002.430514.x"},{"key":"10.1160\/TH16-12-0967-6","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2005.01065.x"},{"key":"10.1160\/TH16-12-0967-7","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12670"},{"key":"10.1160\/TH16-12-0967-8","doi-asserted-by":"crossref","unstructured":"Fischer K, Nijdam A, Holmstrom M, et al. Evaluating outcome of prophylaxis in haemophilia: objective and self-reported instruments should be combined. Haemophilia 2016; Epub ahead of print","DOI":"10.1111\/hae.12901"},{"key":"10.1160\/TH16-12-0967-9","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01312.x"},{"key":"10.1160\/TH16-12-0967-10","doi-asserted-by":"publisher","DOI":"10.1002\/acr.20353"},{"key":"10.1160\/TH16-12-0967-11","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12197"},{"key":"10.1160\/TH16-12-0967-12","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2004.01016.x"},{"key":"10.1160\/TH16-12-0967-13","first-page":"36","volume":"12","author":"van","year":"2006","journal-title":"Haemophilia"},{"key":"10.1160\/TH16-12-0967-14","doi-asserted-by":"publisher","DOI":"10.1160\/TH12-04-0244"},{"key":"10.1160\/TH16-12-0967-15","doi-asserted-by":"publisher","DOI":"10.1191\/026921599668601325"},{"key":"10.1160\/TH16-12-0967-16","doi-asserted-by":"publisher","DOI":"10.1160\/TH15-08-0637"},{"key":"10.1160\/TH16-12-0967-17","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2010.03864.x"},{"key":"10.1160\/TH16-12-0967-18","doi-asserted-by":"publisher","DOI":"10.1007\/s00330-015-4013-8"},{"key":"10.1160\/TH16-12-0967-19","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12587"},{"key":"10.1160\/TH16-12-0967-20","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.1998.440413.x"},{"key":"10.1160\/TH16-12-0967-21","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2004.00903.x"},{"key":"10.1160\/TH16-12-0967-22","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12119"},{"key":"10.1160\/TH16-12-0967-23","doi-asserted-by":"crossref","unstructured":"Poonnoose PM, Hilliard P, Doria AS, et al. Correlating clinical and radiological assessment of joints in haemophilia: results of a cross sectional study. Haemophilia 2016; Epub ahead of print","DOI":"10.1111\/hae.13023"},{"key":"10.1160\/TH16-12-0967-24","doi-asserted-by":"publisher","DOI":"10.1097\/MBC.0000000000000554"},{"key":"10.1160\/TH16-12-0967-25","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02474.x"},{"key":"10.1160\/TH16-12-0967-26","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12134"},{"key":"10.1160\/TH16-12-0967-27","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12366"},{"key":"10.1160\/TH16-12-0967-28","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12755"},{"key":"10.1160\/TH16-12-0967-29","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12290"}],"container-title":["Thrombosis and Haemostasis"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1160\/TH16-12-0967.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,5,6]],"date-time":"2019-05-06T15:37:25Z","timestamp":1557157045000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1160\/TH16-12-0967"}},"subtitle":["Validation of the Haemophilia Joint Health score and Haemophilia Activities List"],"short-title":[],"issued":{"date-parts":[[2017]]},"references-count":29,"journal-issue":{"issue":"08","published-online":{"date-parts":[[2017,11,22]]},"published-print":{"date-parts":[[2017,8]]}},"URL":"https:\/\/doi.org\/10.1160\/th16-12-0967","relation":{},"ISSN":["0340-6245","2567-689X"],"issn-type":[{"type":"print","value":"0340-6245"},{"type":"electronic","value":"2567-689X"}],"subject":[],"published":{"date-parts":[[2017]]}}}