tjh-v38i2

Turk J Hematol 2021;38:101-110
Kavaklı K. et al: Ultrasound and Monitoring for Hemophilia
EQ-5D-3L and SF-36 questionnaires. The QoL scores of the
patients were generally high, indicating a better QoL, and
the mean scores were similar at the baseline and 12 th month.
Assessment of QoL not only gives information about clinical
statuses of patients but also provides an objective criterion
for measuring benefit gained from treatment [19,20]. Better
adherence of patients to treatment is associated with better
outcomes and is a factor that improves QoL [21,22,23].
During the follow-up of PwH, joint function is assessed by
physical examination and the HJHS is widely used for scoring.
However, implementation of the HJHS requires training and
experience [24]. Plain radiographs have also long been used
to evaluate the musculoskeletal system. The Pettersson scoring
system, a radiological joint scoring system, gives quite reliable
outcomes when applied by an experienced radiologist [24].
MRI is a more sensitive imaging method than plain radiogram
Table 6. Comparison of the joint scores at the baseline and 12 th month.
Total scores
All patients
HJHS 52
HEAD-US 52
Baseline
n
Mean ± SD
Median (min-max)
8.98±11.76
3 (0-48)
9.90±9.51
7 (0-33)
12 th month
Mean ± SD
Median (min-max)
11.69±14.93
3 (0-57)
11.37±10.67
8 (0-40)
p
0.007
0.007
Pettersson 13
17.00±16.32
12 (0-47)
17.23±17.14
14 (0-48)
0.631
Children (6-17 years)
HJHS 29
1.86±3.07
1 (0-13)
3.17±7.07
1 (0-36)
0.166
HEAD-US 29
4.03±4.59
2 (0-16)
4.69±4.35
5 (0-14)
0.322
Pettersson 6
3.83±4.45
2.5 (0-11)
2.67±2.66
2.5 (0-7)
0.197
Adults (≥18 years)
HJHS 23
17.96±12.55
17 (0-48)
22.43±15.4
22 (0-57)
0.017
HEAD-US 23
17.3±8.96
18 (0-33)
19.78±10.34
22 (0-40)
0.006
Pettersson 7
28.29±13.94
32 (12-47)
29.71±13.7
32 (14-48)
0.041
Hemophilia A patients
HJHS 42
8.76±11.56
2.5 (0-48)
10.95±13.61
3 (0-48)
0.020
HEAD-US 43
9.35±9.63
6 (0-33)
10.86±10.58
7 (0-40)
0.010
Pettersson 10
18.9±17.91
11.5 (0-47)
18.7±18.99
10.5 (0-48)
0.752
Hemophilia B patients
HJHS 10
9.9±13.16
3.5 (0-36)
14.8±20.15
5 (0-57)
0.232
HEAD-US 9
12.56±8.99
13 (1-30)
13.78±11.4
11 (0-33)
0.338
Pettersson 3
10.67±8.74
13 (1-18)
12.33±9.61
14 (2-21)
0.102
HJHS: Hemophilia Joint Health Score; HEAD-US: Hemophilia Early Arthropathy Detection with Ultrasound; SD: standard deviation; min-max: minimum-maximum.
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