Table 2.
Epidemiological data of Austrian HAE patients as of August 2018
| Characteristics of HAE patients in Austria | N | [%] | ||||||
|---|---|---|---|---|---|---|---|---|
| Total number (as of August 2018) | 137 | 100 | ||||||
| Male | 60 | 43.8 | ||||||
| Female | 77 | 56.2 | ||||||
| Diagnosis | N a | [%] b | ||||||
| HAE‐1 (confirmed) | 77 | 80.2 | ||||||
| HAE‐2 (confirmed) | 19 | 19.8 | ||||||
| Patients without HAE‐1/2 type identification | 41 | |||||||
| Inheritance | N a | [%] | ||||||
| Unrelated HAE families | 27 | |||||||
| Patients with a family history of HAEc | 72 | 83.7 | ||||||
| Patients without a family history of HAEd | 14 | 16.3 | ||||||
| Patients with no information on inheritance | 51 | |||||||
| Age at onset of HAE symptoms [y] | Mean | ±SD | N a | Median | Q1 | Q3 | Min | Max |
| Total | 12.6 | (±13.0) | 40 | 6.5 | 4.75 | 17.25 | 3.0 | 67.0 |
| Male | 12.3 | (±14.6) | 20 | 6.5 | 5.0 | 12.75 | 3.0 | 67.0 |
| Female | 12.9 | (±11.7) | 20 | 9.0 | 4.75 | 17.25 | 3.0 | 51.0 |
| HAE patients born before 1960 | 20.4 | (±18.4) | 13 | 16.0 | 9.0 | 19.0 | 5.0 | 67.0 |
| HAE patients born after 1960 | 8.8 | (±7.4) | 27 | 5.0 | 4.5 | 11.5 | 3.0 | 28.0 |
| HAE patients born after 1980 | 7.6 | (±5.2) | 11 | 6.0 | 5.0 | 13.5 | 3.0 | 51.0 |
| HAE‐1 | 10.7 | (±10.7) | 27 | 6.0 | 5.0 | 13.5 | 3.0 | 51.0 |
| HAE‐2 | 18.4 | (±20.2) | 8 | 13.0 | 9.0 | 16.0 | 4.0 | 67.0 |
| Age at correct diagnosis [y] | Mean | ±SD | N a | Median | Q1 | Q3 | Min | Max |
| HAE patients (total) | 22.9 | (±16.4) | 55 | 21.0 | 8.5 | 31.0 | 0.0 | 69.0 |
| Diagnostic delay [y] | Mean | ±SD | N a | Median | Q1 | Q3 | Min | Max |
| Totale | 15.0 | (±9.9) | 23 | 15.0 | 8.0 | 19.5 | 1.0 | 33.0 |
| HAE patients born before 1960e | 17.6 | (±11.8) | 11 | 16.0 | 9.5 | 29.0 | 1.0 | 33.0 |
| HAE patients born after 1960e | 12.5 | (±7.6) | 12 | 12.0 | 3.0 | 15.75 | 1.0 | 27.0 |
| HAE patients born after 1980e | 7.2 | (±5.9) | 6 | 6.5 | 2.25 | 11.5 | 1.0 | 15.0 |
| Patients with a family history of HAE | 0.5 | (±4.7) | 17 | 0.0 | –1.0 | 0.0 | –6.0 | 16.0 |
a
Number of analyzed patients varies, since only complete and confirmed data have been processed.
b
Percentage refers to patients with confirmed HAE‐1/2 diagnosis (n = 96).
c
Positive family history = more than one affected person in the family (horizontal/vertical, dead or alive).
d
Patients with no affected family member reported in the previous, same or next generation.
e
Calculated on the basis of data from patients without a family history of HAE and data from patients with a family history of HAE who were the first in their families to be diagnosed with HAE.