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Clinical and ultrasound evaluation of patients with haemophilia on prophylaxis
Author(s) -
Daffunchio Carla,
Galatro Gustavo,
Rossi Martín,
Landro María,
Andreu Mauro,
Neme Daniela,
Caviglia Horacio
Publication year - 2021
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1111/hae.14312
Subject(s) - medicine , haemophilia , arthropathy , concordance , subclinical infection , gold standard (test) , physical therapy , elbow , surgery , pediatrics , osteoarthritis , alternative medicine , pathology
Primary prophylaxis is the current gold standard in haemophilia care for the prevention of bleeding and ensuing joint damage. Early detection of joint bleeding, whether symptomatic or subclinical, preferably during childhood, helps prevent joint deterioration and subsequent disability. The aim of this study is to evaluate the level of agreement between the Haemophilia Joint Health Score and the Haemophilia Early Arthropathy Detection with Ultrasound tools in children with severe haemophilia on primary and secondary prophylaxis. Materials and Methods All patients were followed up regularly at our centre. Elbows, knees and ankles were evaluated by physical examination using the Haemophilia Joint Health Score 2.1 (HJHS 2.1), and by ultrasound with HEAD‐US score. Results A total of 80 children with haemophilia on prophylaxis were included in this study. Mean age was 10.8 years (range 4–18). We evaluated 480 joints, of which 423 (88.1%) were concordant with both tools, whereas 57 (11.9%) were discordant; 377 (78.5%) joints scored 0 on HJHS, 370 (77%) on HEAD‐US and 345 (72%) on both tools. The overall Kappa concordance coefficient was .656. For elbows, knees and ankles the respective values were .783, .522 and .589. For HJHS scores greater than 3, all joints scored ≥1 on HEAD‐US. Conclusion HJHS and HEAD‐US are used to assess joint health in children with haemophilia on prophylaxis. In this study, the level of agreement between both tools was consistent with literature values only for the elbow joint.