TABLE 1.
Clinical features of the Japanese patients with HAE‐C1‐INH and HAEnCI
| Variables |
HAE‐C1‐INH % (no. affected/observed a ) |
HAEnCI % (no. affected/observed a ) |
p |
|---|---|---|---|
| Female | 66.9 (105/157) | 95.2 (20/21) | .008 |
| Comorbid disease | |||
| AID | 3.2 (5/158) | 0.0 (0/21) | 1.000 b |
| Urticaria | 7.6 (12/158) | 0.0 (0/21) | .365 b |
| Bronchial asthma | 5.1 (8/157) | 9.5 (2/21) | .334 b |
| CVD | 3.2 (5/158) | 9.5 (2/21) | .192 b |
| Arthralgia/arthritis | 3.2 (5/158) | 0.0 (0/21) | 1.000 b |
| Any kinds | 27.2 (43/158) | 42.9 (9/21) | .138 |
| Family history | |||
| At least another patient in family member | 81.6 (129/158) | 100.0 (21/21) | .027 b |
| Death probably by angioedema | 7.6 (12/158) | 4.8 (1/21) | 1.000 b |
| Age of onset | |||
| Mean ± SD | 23.0 ± 12.6 | 26.8 ± 22.7 | .298 |
| ≤40 | 95.2 (120/126) | 82.4 (14/17) | .075 b |
| ≤20 | 51.6 (65/126) | 52.9 (9/17) | .916 |
| Age of diagnosis | |||
| ≤40 | 69.9 (100/143) | 70.0 (14/20) | .995 |
| ≤20 | 13.3 (19/143) | 40.0 (8/20) | .007 b |
| Site of attacks | |||
| Extremities | 39.2 (62/158) | 47.6 (10/21) | .462 |
| Tongue | 0.6 (1/158) | 4.8 (1/21) | .221 b |
| Face | 32.9 (52/158) | 61.9 (13/21) | .009 |
| Pharynx/Larynx | 21.5 (34/158) | 47.6 (10/21) | .009 |
| Intestine | 35.4 (56/158) | 38.1 (8/21) | .812 |
| Urogenital | 6.3 (10/158) | 9.5 (2/21) | .636 b |
| Others | 7.0 (11/158) | 14.3 (3/21) | .216 b |
| Frequency of attacks in the previous 1 year | |||
| Mean ± SD | 3.66 ± 7.14 | 14.4 ± 27.3 | .0001 |
| ≧6 times | 16.5 (26/158) | 52.4 (11/21) | .0001 |
| Prodromal symptom | 13.3 (21/158) | 28.6 (6/21) | .097 b |
| Trigger | |||
| Psychological | 13.9 (22/158) | 33.3 (7/21) | .051 b |
| Physical | 12.7 (20/158) | 38.1 (8/21) | .007 b |
| Menstruation/Pregnancy | 19.0 (20/105) | 20.0 (4/20) | 1.000 b |
| Dental procedure | 13.9 (22/158) | 14.3 (3/21) | 1.000 b |
| URI | 5.1 (8/158) | 19.0 (4/21) | .037 b |
| Medication and acute attack c | |||
| pdC1‐INH | 31.0 (49/158) | 19.0 (4/21) | .259 |
| Tranexamic acid | 19.0 (30/158) | 38.1 (8/21) | .083 b |
| Anti‐histamine | 6.3 (10/158) | 19.0 (4/21) | .064 b |
| Prednisolone | 3.2 (5/158) | 14.3 (3/21) | .053 b |
| Epinephrine | 1.3 (2/158) | 4.8 (1/21) | .314 b |
| Danazol | 2.5 (4/158) | 0.0 (0/21) | 1.000 b |
| FFP | 0.0 (0/158) | 0.0 (0/21) | – |
| Intubation/Tracheostomy | 3.8 (6/158) | 9.5 (2/21) | .238 b |
| Prophylaxis | |||
| Tranexamic acid | 31.0 (49/158) | 33.3 (7/21) | .829 |
| Danazol | 5.1 (8/158) | 4.8 (1/21) | 1.000 b |
| Others | 6.3 (10/158) | 9.5 (2/21) | .636 b |
A total number of registered are 158 for HAE‐C1‐INH and 21 for HAEnCI.
Abbreviations: AID, autoimmune disease; CVD, cardiovascular disease; FFP, fresh frozen plasma; SD, standard deviation; URI, upper respiratory infection.
a
Some of the data are lacking.
b
Fisher's exact test.
c
Icatibant was approved in Japan after the termination of this registration.