C1 Esterase Inhibitor
Mechanism :
Believed to suppress contact system activation via inactivation of plasma kallikrein and factor XIIa, thus preventing bradykinin production.
Indication :
Contraindications :
History of anaphylactic or life-threatening hypersensitivity reactions to C1 inhibitor (human) or any component of the formulation.
Dosing :
6 to 11 years:
IV 500 units every 3 to 4 days; adjust dose based on individual patient response, up to 1,000 units every 3 to 4 days
Children ≥12 years and Adolescents:
IV 1,000 units every 3 to 4 days; if response is not adequate, doses up to 2,500 units (≤100 units/kg) every 3 or 4 days may be considered based on individual patient response.
HAE attacks (abdominal, facial or laryngeal), treatment:
Children ≥5 years and Adolescents:
IV: 20 units/kg.
Adverse Effect :
Headache, nausea, injection site reactions, nasopharyngitis, dizziness, erythema, pruritus, hypersensitivity reactions, flu-like symptoms.
Interaction :
Androgens: May enhance the thrombogenic effect of C1 inhibitors.
Estrogen Derivatives: May enhance the thrombogenic effect of C1 inhibitors.
Progestins: May enhance the thrombogenic effect of C1 inhibitors.
Hepatic Dose :
No dosage adjustments are recommended.