Findings | Case 1 | Case 2 | Case 3 | Case 4 | Case 5 |
Age at initiation (years) | 8 | 18 | 25 | 8 | 51 |
Gender | F | F | F | M | F |
HFA | Yes | Yes | No | No | Yes |
Hospitalizations | No | Yes | Yes | Yes | Yes |
IOT/UCI | No | Yes | Yes | No | Yes |
Urticaria and/or histamingergic manifestations | No | No | Yes (sun, pineapple, pepper, NSAIDs) | No | Yes (mint, condiments, vegetable products, NSAIDs) |
Complement C4 (mg/dl) | 8.9 | 5.8 | 10 | 6 | 4 |
Complement C4 (2a) | <10 | 6 | 18 | 1.5 | 6 |
C1 inhibitor (mg/dl) (normal 21 - 39 mg/dl) | 4.8 | 11.3 | 5.1 | 0 | 0 |
C1 inhibitor (mg/dl) (confirmed) | 11 | 14.2 | 5.9 | 9.6 | 0 |
HAE type | I | I | I | I | I |
Pharmacological treatment | Danazol LMWH | Danazol LMWH | Danazol LMWH | Danazol LMWH | C1Inh synthetic analogues Antagonists BR2 LMWH |
Adverse treatment effects | None | None | Abnormal uterine hemmorhage | Growth delay | Steatohepatitis Urticaria Anaphylaxis |
Triggers of angioedema | Suspension of danazol Stress Menstrual cycle | Suspension of danazol Infections Stress NSAIDs | Stress NSAIDs High salicylate-containing foods | Stress | Infections Stress NSAIDs High salicylate-containing foods |
Decrease in number of episodes following suspension of NSAIDs and salicylates | No | Yes | Yes | No | Yes |