Validation of a new pediatric joint scoring system from the International Hemophilia Prophylaxis Study Group: Validity of the hemophilia joint health score

Abstract

Objective. Repeated hemarthrosis in hemophilia causes arthropathy with pain and dysfunction. The Hemophilia Joint Health Score (HJHS) was developed to be more sensitive for detecting arthropathy than the World Federation of Hemophilia (WFH) physical examination scale, especially for children and those using factor prophylaxis. The HJHS has been shown to be highly reliable. We compared its validity and sensitivity to the WFH scale. Methods. We studied 226 boys with mild, moderate, and severe hemophilia at 5 centers. The HJHS was scored by trained physiotherapists. Study physicians at each site blindly determined individual and total joint scores using a series of visual analog scales. Results. The mean age was 10.8 years. Sixty-eight percent were severe (93% of whom were treated with prophylaxis), 15% were moderate (24% treated with prophylaxis), and 17% were mild (3% treated with prophylaxis). The HJHS correlated moderately with the physician total joint score (r s ‫؍‬ 0.42, P < 0.0001) and with overall arthropathy impact (r s ‫؍‬ 0.42, P < 0.0001). The HJHS was 97% more efficient than the WFH at differentiating severe from mild and moderate hemophilia. The HJHS was 74% more efficient than the WFH at differentiating subjects treated with prophylaxis from those treated on demand. We identified items on the HJHS that may be redundant or rarely endorsed and could be removed from future versions. Conclusion. Both the HJHS and WFH showed evidence of strong construct validity. The HJHS is somewhat more sensitive for mild arthropathy; its use should be considered for studies of children receiving prophylaxis.

Intermediate-dose versus high-dose prophylaxis for severe hemophilia: comparing outcome and costs since the 1970s

“…16,17 The HJHS is based on physical examination of elbows, knees, and ankles (maximum, 20-26 points per joint) and observation of gait for knees and ankles (0-4 points). The total score was calculated without adding overall global gait to the individual joint scores, resulting in a total score ranging from 0, signifying perfect joint health, to 144.…”

A comparison between MRI, sonography and Functional Independence Score in Haemophilia methods in diagnosis, evaluation and classification of arthropathy in severe haemophilia A and B

“…Radiographic changes depend on the age of the patient at the time of the bleed, the site of the bleed and the acuteness or chronicity of the disease. Sonography may be useful in evaluating joint fluid, synovial hyperemia and cartilage in children with haemophilia [2][3][4][5].…”

Optimal treatment strategies for hemophilia: achievements and limitations of current prophylactic regimens

“…It includes most of the elements of the previously used Gilbert score. [28][29][30][31] Plain x-rays scored by the Pettersson scale had been a useful tool in the past, but are not able to recognize early joint disease. 16,32 MRI is a very sensitive instrument for early detection of hemarthrosis, including synovial hypertrophy, hemosiderin deposition, and osteochondral changes.…”