Treatment
Table 3. Drugs Commonly Used for Long-term HAE Prophylaxis
Drug Name
(Brand)
Usual Adult
Dosage
(Range)
Usual
Pediatric
Dosage
a
(Range)
FDA
Approved/
HAE
Indication Side Effects
17α-alkylated androgens
Danazol
(Danocrine
®
)
200 mg/day
(100 mg q3d -
600 mg/day)
50 mg/day
(50 mg/week -
200 mg/day)
Yes / Yes Common: weight
gain, virilization,
acne, altered libido,
muscle pains and
cramps, headaches,
depression, fatigue,
nausea, constipation,
menstrual abnormali-
ties and increase in
liver enzymes, hyper-
tension, alterations in
lipid profile
Unusual: Decreased
growth rate in chil-
dren, masculinization
of the female fetus,
cholestatic jaundice,
peliosis hepatis
and hepatocellular
adenoma
Stanozolol
(Winstrol
®
)
2 mg/day
(1 mg q3d -
6 mg/day)
0.5 mg/day
(0.5 mg/week -
2 mg/day)
Yes / Yes
Oxandralone
(Oxandrin
®
)
10 mg/day
(2.5 mg q3d -
20 mg/day)
0.1 mg/kg/day
(2.5 mg/week -
7.5 mg/day)
Yes / No
Methyltestosterone
(Android
®
)
Men only:
10 mg/day
(5 mg q3d -
30 mg/day)
Not recom-
mended for
children
Yes / No
Antifibrinolytics
Epsilon
aminocaproic acid
(Amicar
®
)
2 g tid
(1 g bid -
4 g tid)
0.05 g/kg bid
(0.025 g/kg
bid - 0.1 g/kg
bid)
Yes / No Potential: nausea,
vertigo, diarrhea,
postural hypoten-
sion, fatigue, muscle
cramps with increased
muscle enzymes
Unusual: enhanced
thrombosis
Tranexamic acid
(Cyklocapron
®
,
Lysteda
®
)
Available in USA
for PO and IV
administration
1 g bid
(0.25 g bid -
1.5 g bid)
20 mg/kg bid
(10 mg/kg
bid - 25 mg/kg
tid)
Yes / No
a
ere is a relative contraindication for use of these drugs in children and adolescents.
Adapted from Zuraw, BL. N Engl J Med. 2008;359(10):1027-36.
