Indication CINRYZE® (C1 esterase inhibitor [human]) is indicated for routine prophylaxis against angioedema attacks in adults, adolescents and pediatric patients (6 years of age and older) with Hereditary Angioedema (HAE).
In pediatric patients (age 7 to 11), CINRYZE was shown to reduce the number of HAE attacks, lessen the severity of attacks, and reduce the use of acute treatment compared to baseline.1
CINRYZE was shown to reduce the frequency of HAE attacks in 12 pediatric patients (7-11 years old).2
Reduction in frequency of attacks in pediatric patients
Time-normalized mean number of HAE attacks per month
CINRYZE 500 U1.2
CINRYZE 1,000 U0.7
CINRYZE demonstrated a reduction in the number HAE attacks from baseline
71.1% and 84.5% mean reduction in attacks (500 U and 1,000 U, respectively)
Mean absolute reduction in number of attacks: -2.6 (500 U), -3.0 (1,000 U)
A randomized, single-blind, multi-center, dose-ranging cross-over study in 12 pediatric patients with HAE (7 to 11 years old). After an observation period of 12 weeks, patients were randomized to receive either CINRYZE 500 U or CINRYZE 1,000 U every 3 to 4 days for 12 weeks. Patients were then crossed-over to the alternative dose for an additional 12-week period. Efficacy endpoints that were met included reduction in the number of attacks, along with lessened severity of attacks and reduction in the use of acute treatment compared with baseline.1
CINRYZE was proven to reduce the severity of HAE attacks and reduce the use of acute treatment in pediatric patients2
Results from a clinical study in 12 children (7-11 years old) receiving CINRYZE (500 U and 1,000 U)
Average change in the severity of attacks per month vs baseline observationa
-5.2 and -5.8 with 500 U and 1,000 U, respectively
Change in the number of attacks requiring acute treatment per month vs baseline observation
-1.6 and -1.9 with 500 U and 1,000 U, respectively
aSeverity of attacks were measured on a 3-point scale (1=mild, 2=moderate, and 3=severe). Results are the decrease in the total of the symptom severity score for each attack.
Contraindications: CINRYZE is contraindicated in patients who have manifested life-threatening immediate hypersensitivity reactions, including anaphylaxis, to the product.
Hypersensitivity Reactions: Severe hypersensitivity reactions may occur during or after administration of CINRYZE. Consider treatment methods carefully, because hypersensitivity reactions may have symptoms similar to HAE attacks. In case of hypersensitivity, discontinue CINRYZE infusion and institute appropriate treatment. Have epinephrine immediately available for treatment of an acute severe hypersensitivity reaction.
Thromboembolic Events: Serious arterial and venous thromboembolic (TE) events have been reported at the recommended dose of C1 Esterase Inhibitor (Human) products, including CINRYZE, following administration in patients with HAE. Risk factors may include presence of an indwelling venous catheter/access device, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives, certain androgens, morbid obesity, and immobility. Benefits of CINRYZE for routine prophylaxis of HAE attacks should be weighed against the risks of TE events in patients with underlying risk factors. Monitor patients with known risk factors for TE events during and after CINRYZE administration.
Transmissible Infectious Agents: Because CINRYZE is made from human blood, it may carry a risk of transmitting infectious agents, e.g. viruses, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent. ALL infections thought by a physician possibly to have been transmitted by CINRYZE should be reported to Shire Medical Information at 1-800-828-2088.
Adverse Reactions: The only serious adverse reaction observed in clinical studies of CINRYZE was cerebrovascular accident. The most common adverse reactions (≥5%) observed were headache, nausea, rash, vomiting, and fever. Post marketing adverse reactions include local infusion site reactions and hypersensitivity. Post marketing thromboembolic events have been reported, including catheter-related and deep venous thromboses, transient ischemic attack, and stroke.
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To report SUSPECTED ADVERSE REACTIONS, contact Shire Medical Information at 1-800-828-2088 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.