Table 2 Characteristics of international multicenter study (phase I) and the re-test (phase II) sample groups
Characteristics | N total (N/A) | |
|---|---|---|
Phase I | ||
Age (years) (mean ± SD) | 41.5 ± 14.6 | 290 (0) |
Gender (male /female) n (%) | 90 (31) /200(69) | 290 (0) |
C1-INH-HAE type | 270 (20) * | |
Type I, n (%) | 232 (85.9) | |
Type II, n (%) | 38 (14.1) | |
Age at onset of symptoms (years) (mean ± SD) | 11.8 (9.6) | 274 (16) |
Age at HAE diagnosis (years) (mean ± SD) | 26.4 (14.9) | 284 (6) |
Delayed HAE diagnosis (years) (mean ± SD) | 14.4 (13,9) | 271 (19) |
Diagnosis before the onset of symptoms, n (%)* | 11 (4) | 273 (17) |
C1-INH-HAE severity † | 273 (17) | |
Asymptomatic, n (%) | 10 (3.7) | |
Mild, n (%) | 62 (22.7) | |
Moderate, n (%) | 89 (32.6) | |
Severe, n (%) | 112 (41.0) | |
Intubation/tracheotomy requirement ever in life, n (%) | 34 (11.8) | 287 (3) |
Type of residence | 284 (6) | |
Rural / semi-urban (< 25.000 inhabitants), n (%) saaxvdfavefvfvnnnn9()25.000 | 125 (44.0) | |
Urban (≥ 25.000 inhabitants), n (%) | 159 (56,0) | |
Level of education | 290 (0) | |
No schooling/primary school, n (%) | 72 (24,8) | |
High school/further studies, n (%) | 218 (75.2) | |
Socioeconomic level | 280 (10) | |
Low / medium low, n (%) | 173 (61.79) | |
Medium high / high, n (%) | 107 (38.21) | |
Number of emergency visits in the last 6 months | 250 (40) | |
0, n | 149 | |
1–5, n | 70 | |
6–10, n | 16 | |
≥ 11, n | 15 | |
Number of AE attacks in the last 6 months, | 2592 (0) | |
Mean ± SD | 9.3 ± 14.3 | 280 (10) |
Median (range) | 2 (0–111) | |
Maintenance treatment, n (%) | 282 (8) | |
Yes n (%) | 146 (51.8) | |
No n (%) | 136 (48.2) | |
Type of maintenance treatment | 277 (13) | |
Attenuated androgens, yes n (%) | 101 (36.5) | |
Antifibrinolytics, yes n (%) | 18 (6.5) | |
pdC1INH, yes n (%) | 15 (5.4) | |
Others (desogestrel), yes n (%) | 2 (0.7) | |
Several, yes n (%) | 5 (1.7) | |
Inadequate medical treatment of AE episodes in the last 6 months n (%) | 34 (12.3) | 276 (14) |
Psychiatric/psychological care or treatment (in the last 6 months), n (%) | 31 (11.1) | 279 (11) |
pdC1INH at home | 282 (8) | |
No + not available n (%) | 96 (34) | |
Yes + not self-administered n (%) | 143 (50.7) | |
Yes + yes self-administered n (%) | 43 (15.2) | |
Phase II | ||
Age (years) (mean ± SD) | 52.4 ± 13.0 | 20 (0) |
Gender (male /female) n (%) | 10 (50) /10(50) | 20 (0) |
C1-INH-HAE type | 20 (0) | |
Type I, n (%) | 14 (70) | |
Type II, n (%) | 3 (15) | |
Don´t know | 3 (15) | |
Age at onset of symptoms (years) (mean ± SD) | 20.1 (11.7) | 20 (0) |
Age at HAE diagnosis (years) (mean ± SD) | 30.2 (18.7) | 20 (0) |
Family history of C1-INH-HAE, n (%) | 16 (80) | 20 (0) |
Family death from suffocation, n (%) | 5 (25) | 20 (0) |
Intubation/tracheotomy requirement, n (%) | 1 (5) | 20 (0) |