{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T00:51:52Z","timestamp":1787014312446,"version":"build-2736575974"},"reference-count":20,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2022,8,4]],"date-time":"2022-08-04T00:00:00Z","timestamp":1659571200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2022,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Favourable joint outcomes are expected with modern haemophilia A (HA) management. Evaluation of long\u2010term treatment outcomes is hampered by the delay between bleeding episodes during childhood and resulting joint outcomes in adulthood.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>To measure the long\u2010term joint health of adolescents with moderate and severe HA, according to severity and inhibitor status.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Pilot cross\u2010sectional study of five European PedNet centres in moderate and severe HA patients aged 10\u201319 years. Structured assessment of joint status by physical examination (HJHS) and ultrasound (HEAD\u2010US).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>In total, 141 HA patients were evaluable, 100 without inhibitors (81 severe, 19 moderate HA), and 41 severe HA with current\/past inhibitors. On physical examination, 12\/81 (15%) of severe HA without inhibitors, 3\/19 (16%) of moderate HA, and 13\/41 (32%) of severe HA patients with inhibitors exhibited joint abnormalities. Inhibitor persistence, longer inhibitor duration, and a high peak inhibitor level were associated with impaired joint health. Ultrasound showed joint damage (bone or cartilage) in 13\/49 (27%) of severe HA without inhibitors, 1\/12 (8%) of moderate HA, and 10\/28 (36%) of severe HA patients with inhibitors. A discordant ankle evaluation by ultrasound versus physical examination was present in 53\/169 joints (31%).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Most adolescents with severe or moderate HA show favourable joint health. Future research with combined ultrasound and\/or MRI is needed to better understand joint outcomes in the remaining patients. Patents with inhibitors showed a two\u2010fold increased proportion with joint deterioration. Ultrasound paired with physical examination increases sensitivity for detection of joint damage.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hae.14636","type":"journal-article","created":{"date-parts":[[2022,8,4]],"date-time":"2022-08-04T11:18:55Z","timestamp":1659611935000},"page":"1054-1061","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Long\u2010term joint outcomes in adolescents with moderate or severe haemophilia A"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5555-376X","authenticated-orcid":false,"given":"David E.","family":"Schmidt","sequence":"first","affiliation":[{"name":"Childhood Cancer Research Unit Department of Women's and Children's Health Karolinska Institutet  Stockholm Sweden"},{"name":"Astrid Lindgren Children\u00b4s Hospital Karolinska University Hospital  Stockholm Sweden"},{"name":"Division of Hematology Coagulation Unit Karolinska University Hospital  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aikaterini","family":"Michalopoulou","sequence":"additional","affiliation":[{"name":"Hemostasis and Thrombosis Unit Haemophilia Centre Aghia Sophia Children's Hospital  Athens Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7126-6613","authenticated-orcid":false,"given":"Kathelijn","family":"Fischer","sequence":"additional","affiliation":[{"name":"Center for Benign Haematology Thrombosis and Haemostasis Van Creveldkliniek University Medical Center Utrecht Utrecht University  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jayashree","family":"Motwani","sequence":"additional","affiliation":[{"name":"Birmingham Children's Hospital  Birmingham 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Children's Health Karolinska Institutet  Stockholm Sweden"},{"name":"Astrid Lindgren Children\u00b4s Hospital Karolinska University Hospital  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the PedNet Study Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,8,4]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa067659"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01837.x"},{"issue":"1","key":"e_1_2_9_4_1","first-page":"s330","article-title":"Outcome in moderate haemophilia","volume":"12","author":"den Uijl I","year":"2014","journal-title":"Blood Transfus Trasfus 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