tjh-v38i2

Turk J Hematol 2021;38:101-110
Kavaklı K. et al: Ultrasound and Monitoring for Hemophilia
Moreover, joint scores of all patients at the baseline and
follow-up visits are shown in Tables 3-6. When the scores at
the baseline and 12 th month were compared, there were
significant increases in both the HJHS and HEAD-US scores at
the 12 th month in all patients. Separating the patients by age,
no significant increase in any of the scores was observed in
children, but significant increases in the HJHS, HEAD-US, and
Pettersson scores were seen in the adult group.
Evaluation of the patients in terms of hemophilia A and
hemophilia B subgroups showed significant increases in the
HJHS and HEAD-US scores at the 12 th month in the hemophilia A
subgroup (Tables 3 and 6). However, the number of hemophilia B
patients included in this study is much smaller in comparison to
the hemophilia A subgroup. For subgroup analysis by prophylaxis
groups, results are shown in Tables 4 and 5. The elevated scores
found in the adult group were statistically significant compared
to the children.
Correlation analyses were performed for the joint scores at the
baseline and 12 th month. Among all patients, the HJHS total
score was found to significantly correlate with the HEAD-US
total score at the baseline and 12 th month. Moreover, there
was a significant correlation between the Pettersson total score
and the HEAD-US total score at baseline and the 12 th month
(Table 7). Strong correlation was found among the three scoring
systems (HJHS, HEAD-US, and Pettersson) at baseline and after
12 months of observation (p<0.001).
There were no significant differences in the QoL scores between
the baseline and 12 th month in all patients.
Neither pediatric vs. adult group nor hemophilia A vs. hemophilia
B comparisons were significant.
Comparisons of the QoL scores of the patients between the
baseline and 12 th month are presented in Table 8.
Table 3. Joint scores of all patients at baseline and follow-up visits.
Baseline 60
3 rd month 58
6 th month 58
9 th month 53
12 th month 56
n
HJHS
Total score
Mean ± SD
Median (min-max)
9.97±12.41
3 (0-48)
11.1±13.42
5.5 (0-55)
13.95±16.92
5 (0-56)
9.79±13.4
3 (0-50)
11.18±14.55
3 (0-57)
n
61
56
50
41
53
HEAD-US
Total score
Mean ± SD
Median (min-max)
9.70±9.25
7 (0-33)
9.75±9.87
7 (0-36)
9.48±10.41
6.5 (0-40)
9.73±10.31
6 (0-40)
11.51±10.61
8 (0-40)
n
15
- -
- -
- -
13
Pettersson
Total score
Mean ± SD
Median (min-max)
15.4±15.71
11 (0-47)
17.23±17.14
14 (0-48)
HJHS: Hemophilia Joint Health Score; HEAD-US: Hemophilia Early Arthropathy Detection with Ultrasound; SD: standard deviation; min-max: minimum-maximum.
Table 4. Both scoring systems and relationship with frequency of prophylaxis.
HJHS
Total score
Mean ± SD
Median (min-max)
n
Baseline 35
3 rd
month
6 th
month
9 th
month
12 th
month
34
29
28
31
Receiving
prophylaxis
1-2 times a week
11.74±13.32
8 (0-48)
12.06±13.22
6.5 (0-48)
12.14±14.55
4 (0-48)
8.46±12.59
3 (0-48)
10.48±13.44
3 (0-48)
n
15
16
18
16
14
Receiving
prophylaxis
≥3 times a week
5.87±9
2 (0-29)
5.75±6.81
3 (0-25)
11.94±15.52
5.5 (0-54)
10.13±12.09
6 (0-38)
10.79±13.41
5 (0-41)
P
HEAD-US
Total score
Mean ± SD
Median (min-max)
n
0.517 39
0.973 31
0.410 28
0.739 23
0.557 32
Receiving
prophylaxis
1-2 times a week
10.05±10.05
7 (0-33)
10.61±11.45
7 (0-36)
9.46±12
4 (0-40)
9.87±11.25
6 (0-40)
11.44±11.83
7 (0-40)
n
13
16
13
11
11
Receiving
prophylaxis
≥3 times a week
6.69±6.17
5 (0-20)
8.19±6.96
7 (0-22)
8.38±6.05
9 (0-23)
8.27±5.9
8 (0-22)
10.45±6.19
10 (0-22)
HJHS: Hemophilia Joint Health Score; HEAD-US: Hemophilia Early Arthropathy Detection with Ultrasound; SD: standard deviation; min-max: minimum-maximum.
p
0.172
0.244
0.895
0.366
0.795
105