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