Table 1.
Demographic and biological data of patients and controls
| Median (IQR) Range (min – max) |
Sex (F/M) | Age years |
Fg F (g/l) | Fg Ag (g/l) | Fg F/Fg Ag | Hematocrit (L/l) | Platelet (109/l) | ISTH BAT | EXTEM CT (s) |
EXTEM CFT (s) |
EXTEM MCF (mm) |
INTEM CT (s) |
INTEM CFT (s) |
INTEM MCF (mm) |
FIBTEM A10 (mm) |
FIBTEM MCF (mm) |
| Patients Controls |
44/19 22/28 |
40.5 (25.5) 4 – 75 43 (28) 21-63 P = 0.87 |
0.57 (0.295) 0.20 – 1.3 2.64 (2.25) 1.86 – 4.51 P < 0.0001 |
2.7 (1.1) 1.8 – 4.9 |
0.20 (0.09) 0.07–0.66 |
0.41 (0.06) 0.31 – 0.9 0.43 (0.04) 0.37 – 0.51 P = 0.006 |
235 (72) 88 – 404 253 (76) 157 – 379 P = 0.24 |
0 (1) 0 – 5 |
79 (23.5) 40 – 109 73.5 (11.5) 57 – 96 P = 0.095 |
90 (30.5) 37 – 263 77.5 (26) 43 – 148 P = 0.002 |
63 (28) 49 – 77 63.5 (7.25) 55 – 105 P = 0.48 |
191 (217) 114 – 331 236 (125.5) 144 – 427 P = 0.0004 |
98 (108.5) 36 – 188 96 (50.25) 48 – 214 P = 0.54 |
61 (6) (48 – 78) 60 (7) 50 – 134 P = 0.68 |
13 (7.5) 6 – 33 15 (4) 9 – 26 P = 0.018 |
15 (7.5) 7 – 35 17 (5.5) 10 – 29 P = 0.062 |
| FGA | 36/16 | 40.5 (24.5) 7 - 67 |
0.57 (0.25) 0.2 – 1.3 |
2.9 (1.03) 1.9 – 4.9 |
0.19 (0.08) 0.07–0.41 |
0.41 (0.05) 0.31 – 0.7 |
234 (57) (88 – 381) |
0 (1) 0 - 5 |
80.5 (17.75) 40 - 109) |
88.5 (25.5) 37 – 154 |
64.5 (5) 52 – 77) |
191.5 (42.8) 114 - 331 |
91.50 (26.25) 36 - 157 |
64 (4.5) 53 - 78 |
13 (8) 6 – 33) |
15 (8.25) 7 – 35 |
| FGB | 5/3 | 44 (22.75) 30 – 75 |
0.34 (0.36) 0.3 – 0.99 |
2.3 (0.93) 1.8 – 3.8 |
0.16 (0.13) 0.11–0.32 |
0.44 (0.17) 0.39 – 0.9 |
239 (149) 141 - 404 |
1 (1) 0 - 2 |
52 (12.25) 47 - 61 |
147 (62.5) 60 -263 |
55 (6) 49 - 63 |
164 (20.8) 148 - 192 |
126 (50) 64 - 188 |
51.5 (4) 48 - 61 |
15 (4.5) 12 – 20) |
15 (4.25) 13 – 20 |
| FGG | 3/0 | 27 (13.5) 4 – 31 |
1.3 (0.11) 1.1 – 1.3 |
2.1 (0.1) 2.0 – 2.2 |
0.59 (0.07) 0.52–0.66 |
0.4 (0.03) 0.37 – 0.4 |
274 (88) 187 - 275 |
0 (1.5) 0 - 3 |
80 (4.5) 7 – 87 |
135 (8.5) 123 – 140 |
59 (0.5) 58 – 59 |
216 (17) 191 – 225 |
164 (26) 114 – 166 |
56 (1) 54 - 56 |
9 (7) 9 – 23 |
10 (10.5) 10 – 31 |
FGA mutations include Arg35His (n = 46), Gly32Glu (n = 5) and Arg35Cys (n = 1); FGB mutations include Arg196Cys (n = 8); FGG mutations include Tyr306Cys (n = 3).
p, probability from controls and patients’ comparison.