{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T17:26:05Z","timestamp":1788024365519,"version":"build-2784847793"},"reference-count":27,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2013,12,20]],"date-time":"2013-12-20T00:00:00Z","timestamp":1387497600000},"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":[[2014,5]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Haemophilia A is associated with recurrent joint bleeding which leads to synovitis and debilitating arthropathy. Coagulation factor<jats:styled-content style=\"fixed-case\">VIII<\/jats:styled-content>level is an important determinant of bleed number and development of arthropathy . The aim of this study was to compare the haemophilia joint health score (<jats:styled-content style=\"fixed-case\">HJHS<\/jats:styled-content>) and Gilbert score with severity, age, thrombin generation (<jats:styled-content style=\"fixed-case\">TG<\/jats:styled-content>) and underlying mutation in a haemophilia A cohort which had minimal access to haemostatic replacement therapy. Ninety\u2010two haemophilia A individuals were recruited from Pakistan. Age, age at first bleed, target joints, haemophilic arthropathy joints,<jats:styled-content style=\"fixed-case\">HJHS<\/jats:styled-content>and Gilbert score were recorded. A strong correlation was found between<jats:styled-content style=\"fixed-case\">HJHS<\/jats:styled-content>and Gilbert score (<jats:italic>r<\/jats:italic>\u00a0=\u00a00.98), both were significantly higher in severe (<jats:italic>n<\/jats:italic>\u00a0=\u00a059) compared with non\u2010severe (<jats:italic>n<\/jats:italic>\u00a0=\u00a029) individuals before the age of 12\u00a0years (<jats:italic>P<\/jats:italic>\u00a0\u2264\u00a00.01) but not thereafter. When individuals were divided according to developmental age (&lt;12\u00a0years, 12\u201316\u00a0years and &gt;16\u00a0years), both<jats:styled-content style=\"fixed-case\">HJHS<\/jats:styled-content>and Gilbert score were significantly lower in the youngest group (<jats:italic>P<\/jats:italic>\u00a0\u2264\u00a00.001), there was no difference between 12\u201316\u00a0years and &gt;16\u00a0years. In severe individuals there was no correlation between<jats:italic>in vitro<\/jats:italic><jats:styled-content style=\"fixed-case\">TG<\/jats:styled-content>and joint score, whereas in non\u2010severe individuals there was a weak negative correlation. In the severe group, no significant difference was observed for either joint score according to the underlying mutation type (inversion, missense, nonsense, frameshift). In this cohort of haemophilia A individuals with minimal access to haemostatic treatment, haemophilic arthropathy correlated with severity and age; among severe individuals, joint health scores did not relate to either the underlying mutation or<jats:italic>in vitro<\/jats:italic><jats:styled-content style=\"fixed-case\">TG<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/hae.12326","type":"journal-article","created":{"date-parts":[[2013,12,20]],"date-time":"2013-12-20T08:08:47Z","timestamp":1387526927000},"page":"426-434","source":"Crossref","is-referenced-by-count":15,"title":["Joint health scores in a haemophilia A cohort from Pakistan with minimal or no access to factor<scp>VIII<\/scp>concentrate: correlation with thrombin generation and underlying mutation"],"prefix":"10.1111","volume":"20","author":[{"given":"F.","family":"Khanum","sequence":"first","affiliation":[{"name":"Department of Haematology Cardiff University, School of Medicine Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. J.","family":"Bowen","sequence":"additional","affiliation":[{"name":"Department of Haematology Cardiff University, School of Medicine Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B. C.","family":"Kerr","sequence":"additional","affiliation":[{"name":"Department of Haematology Cardiff University, School of Medicine Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P. W.","family":"Collins","sequence":"additional","affiliation":[{"name":"Department of Haematology Cardiff University, School of Medicine Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,12,20]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01312.x"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2796.1994.tb00815.x"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01750.x"},{"key":"e_1_2_8_5_1","first-page":"3","article-title":"Prophylaxis: musculoskeletal evaluation","volume":"30","author":"Gilbert MS","year":"1993","journal-title":"Semin Hematol"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.1999.0050s1016.x"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01307.x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1613195"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0038-1649638"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1159\/000073575"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2008.09.011"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/MBC.0b013e3282f4bb47"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1751-553X.2007.01022.x"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2008.07267.x"},{"key":"e_1_2_8_15_1","first-page":"1","article-title":"Characterization of F8 defects in haemophilia A in Pakistan: investigation of correlation between mutation type and the in vitro thrombin generation assay","author":"Khanum F","year":"2013","journal-title":"Haemophilia"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1159\/000071636"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1186\/1477-9560-3-16"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.20353"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2007.12.016"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01947.x"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01882.x"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa067659"},{"key":"e_1_2_8_23_1","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1111\/j.1365-2516.2009.02178.x","article-title":"Utility of the Haemophilia Joint Health Score in study of episodically treated boys with severe haemophilia A and B in Lithuania","volume":"16","author":"Saulyte Trakymiene S","year":"2010","journal-title":"Haemophilia"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12108"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.5581\/1516-8484.20130010"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-199123000-00006"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2012.02834.x"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2008.03270.x"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.12326","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.12326","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,21]],"date-time":"2024-05-21T23:51:10Z","timestamp":1716335470000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.12326"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,12,20]]},"references-count":27,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2014,5]]}},"alternative-id":["10.1111\/hae.12326"],"URL":"https:\/\/doi.org\/10.1111\/hae.12326","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,12,20]]}}}