Background: The Hemophilia Joint Health Score (HJHS) is an important tool to describe joint status in both pediatric and adult persons with hemophilia (PwH). Age-related, joint-health changes may result in elevated HJHS in healthy adult males, potentially confounding the interpretation of the HJHS in PwH.

Aims: We undertook to define age-related reference ranges for the HJHSv2.1 in healthy adult males.

Methods: Healthy males aged ≥18 years were eligible. Four age-defined strata were prespecified. Subjects with co-morbidities (i.e. rheumatoid arthritis, neurologic disorders) that may independently affect the HJHS were excluded. Subjects with mild sequelae from injury or other mild non-specific degenerative conditions were not excluded. Informed consent was obtained, and the study was approved by recognized ethics committees.

Results: 120 subjects were recruited: 40 aged 18-29; 35 aged 30-40; 22 aged 41-50 and 23 aged >50 years. Median HJHS Total Scores (5^th to 95^th percentile range) (maximum score 124) were 2 (0-9) in those 18-29 years; 3 (0-12) in those 30-40 years; 3 (0-11) in those 41-50 years and 8 (2-19) in those >50 years. Figure 1 shows the boxplot of HJHS Total Score by age category. The heavy line for each box represents the median; the limits of the boxes show the 25^th and 75^th percentiles. HJHS scores increased as a function of age, Spearman´s rho = 0.34 (p < .001). The ankles and knees most often accounted for increased scores. Swelling and crepitus were the most frequent abnormal items, while extension loss and loss of strength were rarely found.

Conclusions: The reference range of the HJHS Total Score in healthy adult males aged < 50 extends up to 12. This data is important for the accurate interpretation of joint outcomes using the HJHS in cohorts of adult PwH.

[HJHS Total Score by Age Category]

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/reference-ranges-of-hjhs-scores-in-healthy-adult-males-without-hemophilia/