{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,8,9]],"date-time":"2024-08-09T19:53:58Z","timestamp":1723233238230},"reference-count":0,"publisher":"Georg Thieme Verlag KG","issue":"S 01","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hamostaseologie"],"published-print":{"date-parts":[[2017]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>\n          Background: Haemophilia is a congenital disorder of coagulation with high economic burden due to its requirement for an expensive, lifelong replacement therapy, with additional costs for the frequent complications and for the severe handicapping consequences. The objective of this cross-sectional study aimed at giving an insight into the health condition of young haemophiliacs in the absence of a regular prophylactic therapy. Methods: It was conducted on a heterogeneous group of 37 children and adolescents (4\u201324 years of age), with similar on demand therapeutic regimen, coming from the whole country, focusing on the joint status by using the Haemophila Joint Health Score (HJHS) system and on quality of life (QoL) by using the EQ-5D-3L-Y questionnaire. Results: The results revealed an impressive situation: 70.3 % with chronic arthropathy, 19 % with target joints, 69 % with multiple joint involvement, mainly elbow (41 %) and knee (34 %), joint damage starting in the age group 6\u201312 years (18.18 % arthropathy vs. 96 % in the age group above 12 years). Joint score (6.67 \u00b1 7.92), gait score (0.75 \u00b1 1.14) and HJHS (7.43 \u00b1 8.78) were highly correlated (r = 0.7, p = 0.001) with the annualised bleeding rate ABR (16.2 \u00b1 12.1). They impacted the QoL in all domains, also expressed by a VAS of 68.39 \u00b1 21.6. Conclusion: We concluded that in the situation of an international consensus that prophylactic replacement can prevent cost-effectively and cost-efficiently the deleterious joint damages, our study is supporting the introduction even of secondary and tertiary prophylaxis in young patients in our country.<\/jats:p>","DOI":"10.5482\/hamo-17-01-0007","type":"journal-article","created":{"date-parts":[[2018,3,20]],"date-time":"2018-03-20T04:33:12Z","timestamp":1521520392000},"page":"S5-S8","source":"Crossref","is-referenced-by-count":1,"special_numbering":"S","title":["A cross-sectional analysis of joint status and quality of life in children and adolescents with haemophilia in Romania"],"prefix":"10.5482","volume":"37","author":[{"given":"Delia","family":"Mihailov","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Smaranda","family":"Arghirescu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dan","family":"Poenaru","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jenel","family":"Patrascu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristina","family":"Ursu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adriana","family":"Diaconu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adina","family":"Traila","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristian","family":"Jinca","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margit","family":"Serban","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2017,12,28]]},"container-title":["H\u00e4mostaseologie"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.5482\/HAMO-17-01-0007.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2018,3,20]],"date-time":"2018-03-20T04:33:13Z","timestamp":1521520393000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.5482\/HAMO-17-01-0007"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017]]},"references-count":0,"journal-issue":{"issue":"S 01","published-online":{"date-parts":[[2017,12,28]]},"published-print":{"date-parts":[[2017]]}},"URL":"https:\/\/doi.org\/10.5482\/hamo-17-01-0007","relation":{},"ISSN":["0720-9355","2567-5761"],"issn-type":[{"value":"0720-9355","type":"print"},{"value":"2567-5761","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017]]}}}