Table 2.
Assessment tool scores for the detection of early structural and functional joint changes
| Physical examination | Structure and function scores | Imaging | Activity and QoL | Participation and QoL | Functional measurement | ||||||||||||
| Parameter | Circ | Pain/ VAS | Gonio | Silent | Gilbert | Colorado | HJHS | Pett | MRI | US | HAL | FISH | SF-36 | HRQoL | MA | EMG | |
| Clinical relevance | Preclinical | 0 | 0 | 1 | 2 | 0 | 0 | 0 | 0 | 3 | 3 | 0 | 0 | 1 | 1 | 3 | 3 |
| Moderate | 1 | 2 | 2 | 2 | 1 | 2 | 2 | 1 | 3 | 3 | 2 | 1 | 2 | 2 | 3 | 3 | |
| Severe | 0 | 2 | 2 | 2 | 2 | 1 | 1 | 2 | 2 | 2 | 3 | 3 | 2 | 2 | 2 | 2 | |
| Content validity | Preclinical | x | 0 | 0 | 3 | 0 | 0 | 0 | 0 | 2 | 2 | 0 | 0 | 0 | 0 | 2 | 2 |
| Moderate | 1 | 1 | 3 | 3 | 1 | 2 | 2 | 1 | 2 | 2 | 2 | 2 | 2 | 2 | 2 | 2 | |
| Severe | x | 1 | 3 | 3 | 2 | 2 | 2 | 1 | 2 | 2 | 2 | 2 | 2 | 2 | 2 | 2 | |
| Sensitivity to change | Preclinical | x | 1 | 0 | 3 | 0 | 0 | 0 | 0 | 3 | 2 | 0 | 0 | 0 | 0 | 3 | 3 |
| Moderate | 1 | 2 | 2 | 3 | 1 | 2 | 2 | 2 | 3 | 2 | 2 | 2 | 2 | 2 | 3 | 3 | |
| Severe | x | 2 | 2 | 2 | 2 | 2 | 2 | 2 | 3 | 1 | 2 | 2 | 2 | 2 | 3 | 3 | |
| Disease specificity | n | n | n | n | y | y | Y | n | n | n | y | y | n | y | n | n | |
| Feasibility | 3 | 3 | 3 | 3 | 3 | 3 | 3 | 2 | 0 | 2 | 3 | 3 | 3 | 3 | 0 | 2 | |
| Suitable for child >3 years | 3 | 1 | 3 | 3 | 1 | 2 | 2 | 2 | 2 | 3 | 2 | 1 | 1 | 1 | 3 | 3 | |
| Structural assessment | y | y | y | y | y | y | Y | y | y | y | n | n | n | n | n | n | |
| Force closure | Preclinical | x | 0 | 0 | 0 | 1 | 1 | 1 | 0 | 0 | 2 | 1 | 1 | 0 | 0 | 2 | 3 |
| Moderate | 0 | 0 | 0 | 0 | 1 | 1 | 1 | 0 | 0 | 2 | 1 | 2 | 0 | 0 | 2 | 3 | |
| Severe | x | 0 | 0 | 0 | 1 | 1 | 1 | 0 | 0 | 2 | 1 | 2 | 0 | 0 | 2 | 3 | |
| Motor control | Preclinical | x | 0 | 0 | 0 | 0 | 0 | 1 | 0 | 0 | 0 | 1 | 1 | 0 | 0 | 3 | 3 |
| Moderate | 0 | 0 | 0 | 0 | 0 | 0 | 1 | 0 | 0 | 0 | 1 | 2 | 0 | 0 | 3 | 3 | |
| Severe | x | 0 | 0 | 0 | 0 | 0 | 1 | 0 | 0 | 0 | 1 | 2 | 0 | 0 | 3 | 3 | |
| Neural control | Preclinical | x | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 1 | 0 | 0 | 3 | 2 |
| Moderate | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 1 | 2 | 2 | 3 | 2 | |
| Severe | x | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 1 | 2 | 2 | 3 | 2 | |
| Activity | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 3 | 3 | 1 | 1 | 0 | 0 | |
| Participation | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 1 | 0 | 2 | 3 | 0 | 0 | |
| Uses | Clinical | 2 | 2 | 2 | 3 | 2 | 2 | 2 | 1 | 3 | 3 | 1 | 3 | 2 | 2 | 3 | 3 |
| Quality control | 0 | 2 | 1 | 2 | 1 | 1 | 2 | 1 | 3 | 3 | 1 | 2 | 2 | 2 | 3 | 3 | |
| Research | 0 | 1 | 0 | 1 | 2 | 2 | 2 | 2 | 3 | 3 | 1 | 2 | 2 | 2 | 3 | 3 | |
| Sum of severity scores | Preclinical | 0 | 1 | 1 | 8 | 0 | 0 | 0 | 0 | 8 | 9 | 2 | 3 | 1 | 1 | 16 | 16 |
| Moderate | 3 | 5 | 7 | 8 | 3 | 6 | 6 | 4 | 8 | 9 | 8 | 10 | 8 | 8 | 16 | 16 | |
| Severe | 0 | 5 | 7 | 7 | 6 | 5 | 5 | 5 | 7 | 7 | 9 | 12 | 8 | 8 | 15 | 15 | |
| Total | 11 | 20 | 24 | 35 | 21 | 24 | 28 | 17 | 34 | 39 | 31 | 39 | 30 | 31 | 59 | 61 | |
Data from [15,17,18,23,26,29,31,43,52,55–78]. The scoring system is described in Table 1; the total score for each tool gives a reflection of its utility across all assessment criteria. Preclinical, moderate and severe relate to stages of arthropathy. Circ, joint circumference; Colorado, Colorado score; EMG, electromyography; FISH, Functional Independence Score in Haemophilia; Gilbert, Gilbert score; Gonio, goniometer; HAL, Haemophilia Activities List; HJHS, Haemophilia Joint Health Score; HRQoL, haemophilia-related quality of life; MA, motion analysis; Pett, Pettersson score; SF-36, 36-item short-form health survey; Silent, Silent symptoms; US, ultrasound; VAS, visual analogue scale.