{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T14:48:47Z","timestamp":1791125327920,"version":"4.1.0"},"reference-count":34,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2026,1,13]],"date-time":"2026-01-13T00:00:00Z","timestamp":1768262400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,1,13]],"date-time":"2026-01-13T00:00:00Z","timestamp":1768262400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>People with haemophilia (PwH) are at increased risk of joint bleeding, often leading to haemophilic arthropathy. The Haemophilia Joint Health Score (HJHS) is widely used to evaluate joint health, but its interpretability is limited by the lack of age\u2010based reference values.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>This study aimed to develop national reference values for the HJHS in individuals with severe haemophilia using data from the Canadian Bleeding Disorder Registry (CBDR).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This cross\u2010sectional study used CBDR data from 1 January 2018 to 31 December 2024. Participants aged \u22654 years with severe Haemophilia A or B and at least one HJHS score were included. Generalised Additive Models for Location, Scale, and Shape (GAMLSS) were used to estimate reference percentiles. Model performance was assessed using 100\u2010fold cross\u2010validation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 551 participants with severe haemophilia were included, comprising 255 paediatric (&lt;18 years of age) and 296 adult patients (mean age: 24\u00a0\u00b1\u00a017 years; range: 4\u201375; 87% with Haemophilia A). Reference percentiles (fifth to 95th) were generated for ages 4\u201370 years, demonstrating a positive association between age and HJHS scores. Cross\u2010validation showed acceptable model consistency.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This study provides the first national age\u2010based reference values for the HJHS in individuals with severe Haemophilia A and B. These values allow clinicians to interpret joint health scores relative to age\u2010specific expectations. Future research should extend age\u2010specific HJHS reference values to additional national registries to strengthen global interpretation and comparison of joint health outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hae.70205","type":"journal-article","created":{"date-parts":[[2026,1,14]],"date-time":"2026-01-14T07:37:15Z","timestamp":1768376235000},"page":"534-542","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Reference Values for the Haemophilia Joint Health Score in Patients With Severe Haemophilia Derived From the Canadian Bleeding Disorder Registry"],"prefix":"10.1111","volume":"32","author":[{"given":"Khang T.","family":"Nguyen","sequence":"first","affiliation":[{"name":"Department of Pediatrics Faculty of Health Science McMaster University  Hamilton Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony K. C.","family":"Chan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Faculty of Health Science McMaster University  Hamilton Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Matino","sequence":"additional","affiliation":[{"name":"Department of Medicine McMaster University  Hamilton Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma","family":"Iserman","sequence":"additional","affiliation":[{"name":"Department of Health Research Methods, Evidence, and Impact McMaster University  Hamilton Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arun","family":"Keepanasseril","sequence":"additional","affiliation":[{"name":"Department of Health Research Methods, Evidence, and Impact McMaster University  Hamilton Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen","family":"Strike","sequence":"additional","affiliation":[{"name":"Hamilton Niagara Regional Hemophilia Program McMaster Children's Hospital Hamilton Health Sciences  Hamilton Ontario Canada"},{"name":"School of Rehabilitation Science Faculty of Health Science McMaster University  Hamilton Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,1,13]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.7326\/M19\u20101208\/SUPPL_FILE\/M19\u20101208_SUPPLEMENT.PDF"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/HAE.13449"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1186\/S12891\u2010021\u201004319\u20100"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/HAE.13117"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/HAE.13858"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/HAE.14046"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ACR.20353"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/RTH2.12690"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/HAE.13631"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/HAE.14812"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/HAE.70012"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rpth.2024.102511"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12891\u2010022\u201006042\u2010w"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-031-50951-3"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/SIM.2227"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/AGEING\/AFAD054"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/HAE.14243"},{"key":"e_1_2_12_19_1","unstructured":"CBDR\u2013AHCDC accessed August 7 2024 https:\/\/www.ahcdc.ca\/cbdr."},{"key":"e_1_2_12_20_1","first-page":"360","volume-title":"Atlas of Diagnostic Hematology, Elsevier","author":"Mitchell W. 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