About Cinryze™ C1 inhibitor (human)
Cinryze™ C1 inhibitor (human) is indicated for routine prophylaxis against attacks of Hereditary Angioedema (HAE), also known as C1 inhibitor deficiency, a disease characterized by unpredictable swelling, which can be dangerous and disfiguring and can cause life-threatening emergencies. Cinryze C1 inhibitor (human) is a sterile, stable, lyophilized preparation of C1 inhibitor derived and manufactured from human plasma, and purified by a combination of nanofiltration and chromatographic procedures.
Cinryze C1 inhibitor (human) Mechanism of Action
C1 inhibitor is a normal constituent of human blood and is one of the serine proteinase inhibitors (serpins). The primary function of C1 inhibitor is to regulate the activation of the complement and intrinsic coagulation (contact system) pathway. C1 inhibitor also regulates the fibrinolytic system. Regulation of these systems is performed through the formation of complexes between the proteinase and the inhibitor, resulting in inactivation of both and consumption of the C1 inhibitor.
HAE patients have low levels of endogenous or functional C1 inhibitor. Although the events that induce attacks of angioedema in HAE patients are not well defined, it is thought by some that increased vascular permeability and the clinical manifestation of HAE attacks are primarily mediated through contact system activation. Suppression of contact system activation by C1 inhibitor through the inactivation of plasma kallikrein and factor XIIa is thought to modulate this vascular permeability by preventing the generation of bradykinin6. Administration of Cinryze C1 inhibitor (human) increases plasma C1 functional, antigenic and C4 levels.
How Cinryze C1 inhibitor (human) is Administered
Cinryze™ C1 inhibitor (human) is administered as an intravenous injection rate of 1 mL per minute over 10 minutes at a dose of 1000 units for routine prophylaxis against HAE attacks. One unit (U) of Cinryze C1 inhibitor (human) corresponds to the mean quantity of C1 inhibitor present in 1 mL of normal fresh plasma. Cinryze C1 inhibitor (human) is a lyophilized preparation available in a single-use vial that contains 500 Units (U) of human C1 inhibitor. Each vial must be reconstituted with 5 mL Sterile Water for Injection, USP (diluent). Two reconstituted vials must be used to make a single, 1000 Unit, dose.
Indication
Cinryze C1 Inhibitor (human) is indicated for routine prophylaxis against angioedema attacks in adolescent and adult patients with Hereditary Angioedema (HAE).
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Dosage
Cinryze C1 inhibitor (human) is for intravenous use only. A dose of 1000 Units Cinryze C1 inhibitor (human) can be administered every 3 or 4 days for routine prophylaxis against angioedema attacks in HAE patients.
Cinryze C1 inhibitor (human) is administered at an injection rate of 1 mL per minute.
Contraindications and Warnings
Cinryze C1 inhibitor (human) is contraindicated in patients who have manifested life-threatening immediate hypersensitivity reactions, including anaphylaxis to the product. Thrombotic events have been reported in association with C1 Inhibitor products when used off-label at high doses.
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