Background
Methods
Identification of patients
Data collection
Data handling
Statistical analysis
Results
Parameter | Frequency / mean (SD) |
---|---|
women / men | 57 / 47 |
mean age (years) | 44 (SD 19.8; range 5 - 80) |
mean age women (years) | 44 (SD 19.8; range 13 - 80) |
mean age men (years) | 41 (SD 19.7; range 5 – 72)) |
age groups | |
≤10 years | 3 |
11 – 20 years | 12 |
21 – 30 years | 17 |
31 – 40 years | 14 |
41 – 50 years | 18 |
51-60 years | 21 |
≥60 years | 19 |
symptom onset | |
mean age symptom onset | 11 (SD 8.2) |
mean age symptom onset women | 12 (SD 7.6) |
mean age symptom onset men | 9 (SD 9.2) |
delay of diagnosisa
| 14 (SD 10.9) |
delay of diagnosisa women | n = 37: 14 (SD 10.3) |
delay of diagnosisa men | n = 21: 14 (SD 12.3) |
HAE variants | |
Type I | 102 |
Type II | 2 |
Frequency and localization of angioedema
Total | Women | Men | ||
---|---|---|---|---|
Patients | 104 | 57 | 47 | |
symptomatic patientsa
| 78 | 45 | 33 | p = 0.563 |
number of angioedema attacks | 1770 | 1290 | 480 | p = 0.015 |
≥1/weekb
| 10 | 8 | 2 | |
≤1/week; ≥ 1/monthc
| 30 | 22 | 8 | |
≤1/month; ≥ 1/yeard
| 38 | 15 | 23 |
women (57) | men (47) | |||
---|---|---|---|---|
n of patients | n of AE | n of patients | n of AE | |
abdomen | 40 (70.%) | 575 | 28 (59.%) | 191 |
extremities | 34 (60 %) | 312 | 27 (57.%) | 118 |
headache | 11 (19 %) | 186 | 5 (11 %) | 43 |
trunk | 15 (26.%) | 89 | 7 (15 %) | 37 |
genitals | 11 (19 %) | 70 | 11 (23.%) | 35 |
face | 14 (24.%) | 41 | 8 (17 %) | 33 |
larynx | 9 (16 %) | 17 | 7 (15 %) | 23 |
Prodromal symptoms and trigger factors
Trigger factor | Number of patients | women | men |
---|---|---|---|
All | 77 | 46 | 31 |
Emotions | 53 | 33 (72 %) | 20 (64 %) |
Trauma / Mechanical trigger | 47 | 27 (59 %) | 20 (64 %) |
Foodstuff / allergies | 33 | 20 (43 %) | 13 (42 %) |
Infection | 21 | 13 (28 %) | 8 (26 %) |
Hormones | 35 | 34 (74 %) | 1 (3 %) |
None | 27 | 11 (24 %) | 16 (57 %) |
Therapy
Pharmacological prophylaxis | women and men | women | men |
---|---|---|---|
tranexamic acid | 10 | 6 (10 %) | 4 (8 %) |
danazol | 26 | 13 (23 %) | 13 (27 % |
IRT | |||
pd C1 - INH | 9 | 7 (12 %) | 2 (4 %) |
Therapy of acute attacks | |||
Icatibant | 12 | 9 (16 %) | 3 (6 %) |
pd C1 – INH | 57 | 32 (56 %) | 25 (53 %) |
Number of Angioedema attacks | ||||||||
---|---|---|---|---|---|---|---|---|
no attacks | ≥1/weeka
| ≥1/month ≤ 1/weekb
| ≥1/year ≤ 1/monthc
| Total n of AE | ||||
Women and men | 104 | 26 | 10 | 30 | 38 | 1770 | ||
Women | All | 57 | 12 | 8 | 22 | 15 | 1290 | |
prophylaxis | None | 38 | 5 | 7 | 18 | 8 | 835 | |
Danazol | 13 | 6 | 1 | 2 | 4 | 143 | ||
TA | 6 | 1 | 0 | 2 | 3 | 48 | ||
IRT | C1-INH | 7 | 0 | 2 | 4 | 1 | 264 | |
men | All | 47 | 14 | 2 | 8 | 23 | 480 | |
prophylaxis | None | 30 | 9 | 1 | 5 | 15 | 216 | |
Danazol | 13 | 4 | 0 | 2 | 7 | 64 | ||
TA | 4 | 1 | 1 | 1 | 1 | 78 | ||
IRT | C1-INH | 2 | 0 | 0 | 1 | 1 | 122 |
Location of attacks | All | Women | no prophylaxis vs. Danazol | Men | no prophylaxis vs. Danazol | women vs. men | ||||
---|---|---|---|---|---|---|---|---|---|---|
All | No prophylaxis | Danazol |
p-values | All | No prophylaxis | Danazol |
p-values |
p-values | ||
104 | 57 | 31 | 13 | 47 | 28 | 13 | ||||
abdomen | 68 (65 %) | 40 (70 %) | 24 (77 %) | 6 (46 %) | p = 0.042 | 28 (59 %) | 15 (53 %) | 9 (69 %) | p = 0.344 | p = 0.063 |
extremities | 61 (59 %) | 34 (60 %) | 20 (64 %) | 5 (38 %) | p = 0.111 | 27 (57 %) | 14 (50 %) | 8 (61 %) | p = 0.491 | p = 0.157 |
trunk | 22 (21 %) | 15 (26 %) | 8 (26 %) | 3 (10 %) | p = 0.849* | 7 (15 %) | 4 (14 %) | 0 | p = 0.288* | p = 0.523* |
face | 22 (21 %) | 14 (24 %) | 8 (26 %) | 2 (6 %) | p = 0.697* | 8 (17 %) | 4 (14 %) | 1 (8 %) | p = 0.486** | p = 0.709** |
genitals | 22 (21 %) | 11 (19 %) | 8 (26 %) | 1 (3 %) | p = 0.242* | 11 (23 %) | 5 (18 %) | 3 (23 %) | p = 0.692* | p = 0.586* |
headache | 16 (15 %) | 11 (19 %) | 4 (13 %) | 3 (10 %) | p = 0.404* | 5 (11 %) | 4 (14 %) | 0 | p = 0.288* | p = 0.509* |
larynx | 16 (15 %) | 9 (16 %) | 6 (19 %) | 1 (3 %) | p = 0.654 | 7 (15 %) | 4 (14 %) | 1 (8 %) | p = 0.486** | p = 0.700** |
Comorbidities
Co-morbidities | All patients | Patients without danazol | Patients on danazol | ||||||
---|---|---|---|---|---|---|---|---|---|
w | m | w | m | w | m | ||||
All | 104 | 57 | 47 | 78 | 44 | 34 | 26 | 13 | 13 |
No comorbidities | 71 68 % | 37 65 % | 34 72 % | 56 72 % | 33 75 % | 26 76 % | 12 46 % | 4 31 % | 8 61 % |
Comorbidities | 33 32 % | 20 35 % | 13 28 % | 19 24 % | 11 25 % | 8 23 % | 14 54 %% | 9 69 % | 5 38 % |
Arterial Hypertension | 23 22 % | 14 24 % | 9 19 % | 11 14 % | 6 14 % | 5 15 % | 12 46 % | 8 61 % | 4 31 |
Dyslipidemia | 6 6 % | 5 9 % | 1 2 % | 0 | 0 | 0 | 6 23 % | 5 38 % | 1 2 % |
DVT | 4 4 % | 1 2 % | 3 6 % | 2 2 % | 0 | 2 6 % | 2 8 % | 1 2 % | 1 |
Depression | 4 4 % | 2 3 % | 2 4 % | 2 2 % | 1 2 % | 1 3 % | 2 8 % | 1 | 1 |
CHD | 4 4 % | 3 5 % | 1 2 % | 4 5 % | 3 7 % | 1 3 % | 0 | 0 | 0 |
CVI | 1 0.9 % | 0 | 1 2.1 % | 0 | 0 | 0 | 1 3.8 % | 0 | 1 |
PAD | 1 1 % | 0 | 1 2 % | 1 1 % | 0 | 1 2.9 % | 0 | 0 | 0 |
Diabetes mellitus | 5 5 % | 4 7 % | 1 2 % | 3 4 % | 2 4 % | 1 3 % | 2 8 % | 2 15 % | 0 |
Epilepsy | 1 1 % | 1 2 % | 0 | 1 1 % | 1 2 % | 0 | 0 | 0 | 0 |
Schizophrenia | 1 1 % | 0 | 1 2 % | 0 | 0 | 1 3 % | 0 | 0 | 0 |
Parkinson’s disease | 2 2 % | 2 3 % | 0 | 1 1 % | 1 2 % | 0 | 1 4 % | 1 | 0 |
Hypothyroidism | 2 2 % | 1 2 % | 1 2 % | 1 % | 1 2 % | 0 | 1 4 % | 0 | 1 |
COPD/Asthma | 2 2 % | 2 3 % | 0 | 2 2 % | 2 4 % | 0 | 0 | 0 | 0 |
Osteoporosis | 2 2 % | 2 3 % | 0 | 2 2 % | 2 4 % | 0 | 0 | 0 | 0 |