5 years (SD 4.3, range 4–17.2 years). The mean HJHS score was 24.5 (SD 14.5, range 5–50). The most affected joints were ankles, followed by knees and elbows. Mean HJHS score in age group I (n = 7) was 11.6 (SD 6.5); in group II (n = 13) the score was significantly higher – mean 31.5 (SD 12.8) (P = 0.0002). Ankles, knees and elbows were significantly more impaired based on the HJHS scores in older patients as compared with younger ones. The HJHS appears to be a useful tool in evaluating musculoskeletal outcome of patients

receiving treatment on-demand. Children ≥10 years of age had significantly higher HJHS scores as a sign of progressing haemophilic arthropathy. We conclude that the most aggravating development of haemophilic joint damage seems to occur from the age of 10 and onwards. “
“Summary.  Episodic treatment of bleeding selleck disorders is defined as utilization of clotting factor

concentrates in response to acute bleeding episodes to achieve haemostasis. Non-adherence to prescribed episodic regimens can limit treatment effectiveness and result in target joint formation, FDA approved Drug Library research buy pain and disability. Evaluation of and interventions to promote adherence may improve health outcomes. The purpose of this study was to validate a new adherence scale developed for individuals with bleeding disorders treated on episodic infusion regimens, entitled VERITAS-PRN [Validated Hemophilia Regimen Treatment Adherence Scale – PRN]. Participants were recruited from the Indiana Hemophilia and Thrombosis Center patient population. Participants completed the scale for psychometric development and analysis. Subjective ratings of adherence from participants and providers were used for validation. The study sample

included 51 male and three female patients. Twenty-seven participants (50.0%) were diagnosed with FVIII deficiency, 21 (38.9%) with FIX deficiency and six (11.1%) with von Willebrand’s disease (VWD). Internal consistency reliability for the total VERITAS-PRN score and the majority of subscales was good-to-excellent, with the one exception being the ‘Plan’ subscale. Test-retest reliability correlations were good-to-excellent for Molecular motor the total scale and all subscales. The VERITAS-PRN total scale had moderate-to-strong and statistically significant correlations with validity measures. The VERITAS-PRN is a reliable and valid measure of adherence to episodic treatment regimens for bleeding disorders. This tool may be utilized as a standard measure of adherence to increase sensitivity to adherence problems and promote targeted interventions to enhance adherence and health outcomes “
“Summary.  Children with inherited bleeding disorders often require central venous catheters (CVCs). Although CVCs are known to be complicated by deep venous thrombosis (DVT), little is known about the timeline of DVT development or risk of post-thrombotic syndrome (PTS).