Furthermore, it is not well adapted for patients on prophylaxis with low joint damage (relatively insensitive to mild joint changes) but has some interest in severely affected patients, for example, in countries with limited access to factor replacement therapy. It has been tested on children in North America and Europe with mild, moderate and severe haemophilia A and B, both with and without prophylaxis, but has not really been validated. The HJHS exists in three versions and has an excellent reliability . It takes quite a long time to do (45�C60?min) but needs no special equipment (goniometer, stairs). It involves training. The range of motion measurements should be interpreted according to reference values and their age-related variations . It is available in four languages (English, Swedish, Dutch and Chinese Mandarin). The new version of the score (HJHS 2.1) provides a total score (maximum?=?124, the higher Verteporfin
being the worst), joint-specific scores and a global gait score that is a recent improvement. It is more sensitive than the Gilbert score and is sensitive enough to detect early signs of joint damage. Therefore, it can be used for monitoring joint change over time even in PWH on prophylaxis. It has been tested on children in North America and Europe (usually on prophylaxis with mild joint impairment) and in Chinese boys with moderate-to-severe arthropathy [5-7]. It has been validated in its first version  as well as in children . It has not yet been adequately studied in adults, PWH with severe joint disease or in children aged Selleckchem CH5424802
<4?yr old. HJHS correlates quite well to WFH score (but is 63�C97% more efficient for the discrimination <a href="http://www.selleckchem.com/products/VX-770.html
">VX-770 concentration of known groups) and has a quite good correlation with cumulative number of haemarthroses. Furthermore, it seems to correlate highly with radiographic damage . Details of studies cited in this section are displayed in Table?1. Radiological imaging is used to diagnose, objectively evaluate, monitor and perform a staging of complications of haemophilia, especially arthropathy due to recurrent joint bleeding. The main imaging techniques evaluated in PWH are conventional radiography (X-ray), MRI and ultrasonography (US). X-ray, to analyse bone lesions, has been used for many years to evaluate joint damage in PWH. It is useful to monitor advanced stages but insensitive for early changes of haemophilic arthropathy involving soft tissues or first steps of cartilage destruction. Two main classification systems have been proposed for grading the haemophilic arthropathy: the Arnold�CHilgartner system (progressive scale, simple and easy to use) and the Pettersson's score (additive scale, more difficult to perform) [11-13]. Both scores have good intra- and interobserver variability and demonstrate a quite good correlation in the presence of absence or huge joint changes but poor agreement in cases of mild or moderate arthropathy .