Table 3: Assessment on pediatric use for the treatment of HAE-C1-INH.

Active ingredient/trade name	Risks and adverse events
Human pdC1-INH concentrates
Berinert	Pediatric data for Berinert indicate that is efficacy and safety of 20U/kg in pediatric patients are comparable with that for adults
Cinryze	Pediatric data for Cinryze indicate that its efficacy, safety and tolerability in pediatric patients are comparable with that for adults. The recommended dose (1000 U) is the same for all types of attacks and for all body weigths. The paucity of data on body weight-based dosing, as is otherwise common in pediatric, is seen as critical. No adverse effects in adults and adolescents from high doses have become known to date.
Recombinat human C1-INH concentrate
Ruconest	Until data on children and adolescents are published and approval of the drug is granted, Ruconest should only be used when specifically warranted.
Kallikrein and bradikinin receptor antagonist
Icatibanr/Firazyr	On a preliminary basis, no recommendation favoring use in children and adolescents can be made, given the lack studies in pediatric patients.
Ecallantide/Kalbitor	The currently available data on the treatment of pediatric patients is insufficient. Moreover, no European approval is available.
Attenuated androgens
Danazol/Danatrol	Given their adverse reaction profile, at present, androgens should not be used for
Danazol/Danokrin	The long-term propylaxis of pediatric HAE-C1-INH. Neither can a recommendation
Danazol/Danokrine	be given for short-term prophylaxis, despite the fact that danazol is approved in
Stanozolol/Winstrol	Switzerland for children < 12 years of age
Antifibrinolitics
Tranexamic acid/Cylokapron	The use of Ԑ-aminocaproic acid cannot be recommended, as it is approved in some countries by regulatory authorities for the treatment of HAE. Due to the doubtful efficacy of tranexamic acid, it should be avoided for short-term and long-term prophylaxis in pediatric patient.