Abstract Number: PB1032
Meeting: ISTH 2020 Congress
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 (5th to 95th 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 25th and 75th 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]
To cite this abstract in AMA style:St-Louis J, Abad A, Akins S, Austin S, Chowdary P, Classey S, Funk S, Hernandez G, Hilliard P, Manco-Johnson M, Mangles S, McLaughlin P, Nugent D, Shapiro A, Srivastava A, Tilak M, Wells A, Zourikian N, Blanchette V, Feldman BM. Reference Ranges of HJHS Scores in Healthy Adult Males without Hemophilia [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/reference-ranges-of-hjhs-scores-in-healthy-adult-males-without-hemophilia/. Accessed September 29, 2023.
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