Clinical and ultrasound assessment tools generally agree in their determinations of joint health for children with hemophilia on prophylaxis, although agreement varies by joint, according to research published in Haemophilia.

Prophylaxis is the gold standard for severe hemophilia to prevent bleeding and joint damage. Along with prophylaxis, early detection is needed to prevent joint deterioration and disability.

The Haemophilia Early Arthropathy Detection with ultrasound (HEAD-US) is a systematic assessment of joint status. It was designed for those who are not radiologists to easily and quickly assess joints with ultrasound.

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The Haemophilia Joint Health Score (HJHS) is a physical examination tool to assess joint health in children with hemophilia. Both tools have been validated on their own, but little data exists as to the agreement between the 2.

The study authors sought to determine the level of agreement between these 2 measures of joint health in children receiving primary and secondary prophylaxis. The single-center study included 80 patients aged 4 to 18 years with severe hemophilia receiving prophylaxis. No patients reported bleeding within 2 week before the study assessment.

A total of 34 (42.5%) patients scored 0 with both tools, 33 patients (41.3%) scored 1 or less with both assessments, and 13 patients (16.2%) had discordant results. A total of 480 joints were assessed with both tools. Of the joints analyzed, 88.1% were concordant with both tools. The Kappa concordance coefficient was .783 for elbows, .522 for knees, and .589 for ankles.

Overall, most joints had consistent scores with both assessment tools. There was a 12% discordance found in this study, which is in between the discordance found in other studies. The study authors found the most discrepancies in ankle joints, which is consistent with similar studies.

HEAD-US found joint abnormalities even when the physical examination was normal. Of the total joints assessed that had abnormalities, 8.5% were normal with a physical examination, and 5.5% scored 1 to 3 on HJHS. HJHS scores from 1 to 3 correlate with a loss of range of motion, muscle atrophy, and decreased muscle strength.

The authors concluded that the level of agreement between HEAD-US and HJHS was consistent with literature values (.7) only for the elbow joint. A limitation of the study was that most joints evaluated with this patient population scored low with both tools. It’s unknown if the level of agreement would be different in patients with higher scores.


Daffunchio C, Galatro G, Rossi M, et al. Clinical and ultrasound evaluation of patients with haemophilia on prophylaxis. Haemophilia. Published online May 10, 2021. doi:10.1111/hae.14312