Study of C1 Inhibitor (Human) for the Prevention of Angioedema Attacks and Treatment of Breakthrough Attacks in Japanese Subjects With Hereditary Angioedema (HAE)

Inclusion Criteria

• Be of Japanese descent, defined as born in Japan and having Japanese parents and Japanese maternal and paternal grandparents.
• Be ≥2 years of age.
• Meet the following minimum body weight criteria:
• Subjects 2 to 5 years of age must weigh at least 12.5 kg; and
• Subjects 6 years of age and above must weigh at least 25 kg.
• Have a confirmed diagnosis of Type I or Type II HAE. NOTE: Diagnosis may be based on historical data including family history, clinical symptoms (characteristic attacks), or documentation of low level of C1 INH protein and/or C1 INH activity.
• Have a history of at least one angioedema attack per month (on average) during the 3 consecutive months immediately before enrollment.
• Agree to adhere to the protocol-defined schedule of assessments and procedures.
• Agree to avoid his/her known angioedema attack triggers during the study to the best of his/her ability.
• If a female of reproductive age, be postmenopausal (≥12 months following cessation of menstruation), surgically sterile, or following an acceptable method of birth control (and agree to continue its use through 1 month after the last dose of study drug):
• Non-hormonal methods (eg, abstinence, barrier control) for at least 1 complete menstrual cycle before the Screening Visit.
• Stable doses of estrogen and/or progestin containing products for at least 2 months before the Screening Visit.
• If a male of reproductive age, be surgically sterile or agree to follow an acceptable method of birth control (eg, abstinence, barrier control) from the Screening Visit through 2 months after the last dose of study drug.
• If an adult, be informed of the nature of the study and provide written informed consent before any study-specific procedures are performed.

OR If a child or minor (<20 years of age), have a parent/legal guardian who is informed of the nature of the study provide written informed consent (ie, permission) for the child to participate in the study before any study-specific procedures are performed. Assent will be obtained from children ≥14 years of age.

Exclusion Criteria

• Have a history of hypercoagulability (abnormal blood clotting).
• Have a diagnosis of acquired angioedema or be known to have C1 INH antibodies.
• Have a history of allergic reaction to C1 INH products, including CINRYZE (or any of the components of CINRYZE) or other blood products.
• Have received C1 INH therapy or any blood products within 3 days before the first dose of study drug.
• Have had signs or symptoms of an angioedema attack within 2 days before the first dose of study drug.
• Have any change (start, stop, or change in dose) in androgen therapy (eg, danazol, oxandrolone, stanozolol, testosterone), tranexamic acid, epsilon-aminocaproic acid (EACA), or other antifibrinolytics within 14 days before the first dose of study drug.
• If female, have started taking or changed the dose of any hormonal contraceptive regimen or hormone replacement therapy (eg, estrogen/progestin containing products) within 2 months before the first dose of study drug.
• Be pregnant or breastfeeding.
• Have received an investigational drug other than those required for prevention or treatment of angioedema attacks within 30 days before the first dose of study drug.
• Have, as determined by the Investigator and/or the Sponsor's Medical Monitor, any surgical or medical condition that could interfere with the administration of study drug or interpretation of study results.