Approved October 10, 2008

C1 ESTERASE INHIBITOR, HUMAN
(FDA Category 1S)

CINRYZE (Lev Pharmaceuticals, Inc) is one of the serine proteinase inhibitors in human blood. It regulates the activation of the complement and intrinsic coagulation (contact system) pathways and the fibrinolytic system. C1 inhibitors may reduce the vascular permeability by preventing bradykinin generation activated through the contact system.

DOSING INFORMATION: The recommended dose is 1000 units intravenously (IV) over 10 minutes every 3 to 4 days.

PHARMACOKINETICS: Initial response after IV infusion of C1 inhibitor, human in adult and adolescent patients with hereditary angioedema is 1 hour or less with a time to peak concentration of 3.9 hours. Mean terminal half-life is 56 hours. Total clearance is 0.85 mL/min. It is inactivated through complex formation with the proteinase.

CAUTIONS: Common adverse reactions observed at a rate greater than or equal to 5% were upper respiratory tract infections, sinusitis, rash, and headache. Because C1 esterase inhibitor is made from human blood, there is risk of transmission of infectious viruses.

FDA APPROVED INDICATIONS: C1 inhibitor, human is indicated for routine prophylaxis of angioedema attacks in adults and adolescents with hereditary angioedema.