Table 2.

Therapeutic options—license status

Drug	Registration	Indication				Age/Groups		a
		Acute treatment	Prophylaxis		Home therapy
			STP	LTP		Childrenb	Adolescencec
pdC1‐INH (Berinert®)	Europe	✓	✓	—	✓	✓	✓	i.v.
	USA	✓	—	—	✓	✓	✓
	Latin America (Brazil, Argentina, Mexico, Colombia, Chile, Puerto Rico)	✓	✓	—	✓	✓	✓	i.v.
	Australia	✓	—	—	✓	—	✓	i.v.
	Canada	✓	—	—	—	—	✓	i.v.
	Israel	✓	✓	—	✓	✓	✓	i.v.
	Japan	✓	✓	—	✓	—	✓	i.v.
	South Korea	✓	✓	—	✓	✓	✓	i.v.
pdC1‐INH (Cinryze®)	Europe	✓	✓	✓	✓	Trial ongoing	✓	i.v.
	USA	—	✓	✓	✓	Trial ongoing	✓
	Latin America	—	—	—	—	—	—	i.v.
	Australia	—	✓	✓	✓	—	—	i.v.
	Canada	—	✓	✓	✓	—	—	i.v.
	Israel	✓	✓	✓	✓	—	—	i.v.
rhC1‐INH (Ruconest®)	Europe	✓	—	—	—	Trial ongoing	✓	i.v.
	USA	✓	—	—	—	Trial ongoing	✓
	Latin America	—	—	—	—	—	—
Icatibant (Firazyr®)	Europe	✓	—	—	✓	Trial ongoing	Trial ongoing	s.c.
	USA	✓	—	—	✓	Trial ongoing	Trial ongoing
	Latin America (Brazil, Argentina, Mexico, Colombia)	✓	—	—	✓	—	—	s.c.
	Australia	✓	—	—	✓	Trial ongoing	Trial ongoing	s.c.
	Canada	✓	—	—	✓	—	—	s.c.
	Israel	✓	—	—	✓	Trial ongoing	Trial ongoing	s.c.
	Kuwait	✓	—	—	✓	—	—	s.c.
	South Africa	✓	—	—	✓	—	—	s.c.
Attenuated androgensd	Europe	—	✓	✓	—	—	✓	Oral
	USA	—	—	✓	—	—	—
	Latin America (Brazil, Argentina, Mexico, Colombia)	—	—	✓	✓	—	—	Oral
	Australia	—	—	✓	—	—	—	Oral
Tranexamic acid (Cyklokapron®; Transamin®; Hemoblock®)	Europe	—	—	✓	—	✓	✓	Oral
	USA	—	—	✓	—	✓	✓	Oral
	Canada	—	—	✓	—	✓	✓	Oral
	Australia	—	—	✓	—	✓	✓	Oral
	Latin America (Brazil, Argentina, Mexico, Colombia)	✓	—	—	✓	—	—	Oral
Ecallantide (Kalbitor®)	Europe	—	—	—	—	—	—	s.c.
	USA	✓	—	—	—	—	✓
	Latin America	—	—	—	—	—	—

^ai.v., intravenous; s.c., subcutaneous.

^bChildren aged 0 to ≤12 years.

^cAdolescents aged 12 to ≤18 years.

^dAttenuated androgens not licensed in Germany, Austria, and Switzerland.

pdC1‐INH, human plasma‐derived C1‐INH; rhC1‐INH, recombinant human C1‐INH.