Utility of the Haemophilia Joint Health Score in study of episodically treated boys with severe haemophilia A and B in Lithuania

Abstract

Musculoskeletal outcome remains the major hallmark of haemophilia. The purpose of the study was to assess joint status using a new musculoskeletal assessment tool in children with haemophilia and describe the development of haemophilic arthropathy during childhood and puberty focussing on the age of remarkable changes. The prospective study involved Lithuanian patients aged 4-17 years with severe haemophilia A and B, no signs of inhibitors and treatment on-demand. Patients were subdivided into two groups according to actual age. Group I patients were 4-9 years and group II patients 10-17 years of age. The musculoskeletal status was measured using the Haemophilia Joint Health Score (HJHS). We report on 20 patients with a mean age of 11.5 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 > or =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.

Using routine Haemophilia Joint Health Score for international comparisons of haemophilia outcome: standardization is needed

“…In patients on the most intensive regimen (‘full’ regimen), joint changes measured by HJHS only appeared after the age of 13 years . In patients treated on demand, HJHS was already increased before the age of 10 years, and afterwards significant worsening of HJHS was observed . It therefore seems likely that larger differences between treatment groups, such as when comparing prophylaxis and on demand treatment , would have been picked up despite inter‐observer variability.…”

Clinical and functional evaluation of the joint status of hemophiliac adults at a Brazilian blood center

“…It is still widely used (16) because it is easy to perform and capable of providing an extensive musculoskeletal assessment (4) . A tape measure and a goniometer are used in the examination of the knees, ankles, elbows, and hips for the presence of edema, atrophy, crepitus, flexion contractures, axial deformities, loss of range of motion and instability; points are assigned to each finding, according to severity and are summed.…”

Clinical and ultrasound joint outcomes in severe hemophilia A children receiving episodic treatment in Indonesian National Hemophilia Treatment Center

“…However, earlier studies showed that this measurement also applicable for episodically treated patients. 15,16 Study in Lithuania 16 reported HJHS in 20 hemophilia children receiving episodic treatment, mean total scores in patients aged 4-9 years was significantly lower compare to older children aged 10-17 years (11.6 vs 31.5, respectively). Study in Pakistan 15 on severe (n=59) and non-severe (n=29) hemophilic patients receiving episodic treatment divided into three developmental age groups (<12 years, 12-16 years, and >16 years), the HJHS and Gilbert scores were significantly lower in the youngest group (p<0.001).…”