Table 2: Specific risks and adverse effects of products for the treatment of HAE-C1-INH.

Active ingredient/trade name

Risks and adverse events

Human pdC1-INH concentrates

 

Berinert

 

A theorical risk of pathogen transmission is associated with all plasma products. No such transmissions had been described. In this respect, the product can be judged to be safe. It was speculated that long-term use of Berinert may be associated with an increased frequency of HAE-C1-INH attacks.

Cinryze

 

A theorical risk of pathogen transmission is associated with all plasma products. No such transmissions hace thus far been described. In this respect, the product can be judged to be safe.

Recombinat human C1-INH concentrate

 

Ruconest

 

Several patiens developed antibodies to rabbit antigens (from dander and hair, not from the C1-INH); allergic reactions were observed rarely.

Kallikrein and bradikinin receptor antagonist

 

Icatibanr/Firazyr

 

From the theorical perspective, caution is advised in patients with ischemic heart disease, unstable angina pectoris, and in the first weeks following a stroke. Clinically relevant problems in this regard have not been observed to date.

Ecallantide/Kalbitor

 

Worth mentioning in the risk of anaphylactic reactions (frequency according to the boxed warning in the USA full precribig information, 3,9%).

Attenuated androgens

 

Danazol/Danatrol

Danazol/Danokrin

Danazol/Danokrine

Stanozolol/Winstrol

The most common adverse effects are virilization, weighy gain, menstrual irregularities, depression/aggression, mialgia and acné. Adverse effects such as hypercholesterolemia, hypertension, erythrocytosis and hepatic tumors mandate the need for regular medical checkups. Furthermore, in children, growth disorders and premature closure of the epiphysial cartilage are conceivable and nave not been sufficiently analyzed in clinical sudies.

Antifibrinolitics

 

Tranexamic acid/Cylokapron

The most common adverse effects are dose-dependent gastrointestinal symptoms (nausea, vomiting and diarrea).