Table 1. Clinical features of Slovenian patients with HAE.
Family | Age (years) | Gender | Age at onsetof symptoms | HAE diagnosis (type) | Clinicalseverity score | Skin oedema | Facial oedema | Abdominal oedema | Laryngeal oedema | Prophylactic treatment | Family history | C1-INH, g/l | C1-INH function, % | C4, g/l |
1 | 28 | M | 3 | Type I | 10 | + | – | – | – | Danazol | + | 0.09 | 19 | 0.15 |
1 | 62 | M | 25 | Type I | 5 | + | – | – | – | None | + | <0.05 | 26 | 0.09 |
1 | 83 | F | ND1 | Type I | ND | + | + | – | – | None | – | 0.06 | 53 | 0.14 |
2 | 38 | F | 21 | Type I | 5 | + | + | + | + | Danazol | + | 0.05 | 4 | 0.09 |
2 | 73 | M | Asympt | Asympt | ND | – | – | – | – | None | + | <0.05 | 26 | 0.05 |
3 | 19 | F | 13 | Type II | 2 | + | – | – | – | None | + | 0.56 | 28 | <0.05 |
3 | 23 | M | 12 | Type II | 2 | + | – | – | – | None | + | 0.59 | 17 | <0.05 |
3 | 47 | M | 17 | Type II | 4 | + | + | – | + | Danazol | + | 0.66 | 15 | 0.09 |
4 | 42 | M | 20 | Type I | 4 | + | + | + | – | None | + | <0.05 | 1 | <0.05 |
4 | 77 | F | 21 | Type I | 5 | + | – | – | + | None | + | <0.05 | 32 | <0.05 |
5 | 49 | F | 19 | Type I | 7 | + | – | – | – | None2 | + | <0.05 | 1 | 0.06 |
5 | 30 | M | 16 | Type I | 6 | + | – | – | + | Danazol | + | <0.05 | 19 | <0.05 |
5 | 84 | F | ND1 | Type I | ND | ND | ND | ND | ND | None | – | <0.05 | 1 | <0.05 |
6 | 44 | M | 34 | Type II | 6 | + | + | + | + | None | + | 0.25 | 64 | <0.05 |
6 | 63 | F | 7 | Type II | 5 | + | + | + | – | None | + | 0.31 | 9 | 0.13 |
7 | 40 | F | 16 | Type I | 7 | + | + | + | + | Danazol | + | 0.08 | 33 | <0.05 |
7 | 65 | M | 10 | Type I | 7 | + | + | + | + | Danazol | – | 0.06 | 22 | 0.05 |
8 | 41 | F | 40 | Type II | 1 | – | + | – | – | Danazol | – | 0.25 | 11 | 0.15 |
Asympt: Asymptomatic; ND: Not determined.
Patients have reported angioedema attacks in past but exact time, intensity and onset is unknown.
Side effects of Danazol use.