C1-esterase inhibitor, human


Generic Medicine Info
Indications and Dosage
Intravenous
Hereditary angioedema
Adult: Treatment of acute attacks: As 50 international units/mL: 20 international units/kg via slow IV inj at 4 mL/min. As 100 international units/mL: 1,000 international units via slow IV inj at 1 mL/min, given at the 1st sign of the onset of the attack. Administer a 2nd dose of 1,000 international units if there is no adequate response after 1 hour; if the treatment is intended for acute laryngeal angioedema attacks or there is a delay in treatment initiation, the 2nd dose may be given sooner than 1 hour. Prophylaxis of angioedema attacks: As 100 international units/mL: 1,000 international units via slow IV inj at 1 mL/min every 3 or 4 days. If there is inadequate response, may consider giving 2,000 international units (Max: 80 international units/kg) every 3 or 4 days based on individual patient response. Dosage and treatment recommendations may vary among countries and individual products (refer to specific product guidelines).
Child: Treatment of acute attacks: As 50 international units/mL: Same as adult dose. As 100 international units/mL: 2-11 years weighing 10-25 kg: 500 international units via slow IV inj at 1 mL/min, given at the 1st sign of the onset of the attack. Administer 2nd dose of 500 international units if there is no adequate response after 1 hour; >25 kg: 1,000 international units via slow IV inj at 1 mL/min, given at the 1st sign of the onset of the attack. Administer a 2nd dose of 1,000 international units if there is no adequate response after 1 hour. 12-17 years Same as adult dose. Prophylaxis of angioedema attacks: As 100 international units/mL: 6-11 years 500 international units via slow IV inj at 1 mL/min every 3- or 4 days. If there is inadequate response, may consider giving 1,000 international units every 3 or 4 days based on individual patient response. 12-17 years Same as adult dose. Dosage and treatment recommendations may vary among countries and individual products (refer to specific product guidelines).

Intravenous
Preventive perioperative treatment for hereditary angioedema
Adult: As 50 international units/mL: 1,000 international units via slow IV inj at 4 mL/min, given <6 hours before surgical procedure. As 100 international units/mL: 1,000 international units via slow IV inj at 1 mL/min, given within 24 hours before surgical procedure. Dosage and treatment recommendations may vary among countries and individual products (refer to specific product guidelines).
Child: As 50 international units/mL: 15-30 international units/kg via slow IV inj at 4 mL/min, given <6 hours before surgical procedure. As 100 international units/mL: 2-11 years weighing 10-25 kg: 500 international units via slow IV inj at 1 mL/min, given within 24 hours before surgical procedure; >25 kg: Same as adult dose. 12-17 years Same as adult dose. Dosage and treatment recommendations may vary among countries and individual products (refer to specific product guidelines).

Subcutaneous
Prevention of recurrent attacks of hereditary angioedema
Adult: As 500 international units/mL: 60 international units/kg twice weekly (e.g. every 3-4 days) via SC inj, preferably at the stomach. Dosage and treatment recommendations may vary among countries and individual products (refer to specific product guidelines).
Child: As 500 international units/mL: ≥8 years Same as adult dose. Dosage and treatment recommendations may vary among countries and individual products (refer to specific product guidelines).
Reconstitution
Reconstitute only with the provided solvent and transfer device (refer to specific product guidelines for the detailed instructions for reconstitution).
Contraindications
History of anaphylaxis or potentially fatal hypersensitivity reactions to C1 esterase inhibitor, human.
Special Precautions
Patient with laryngeal oedema; known risk factors for thromboembolic events (e.g. history of thrombosis, atherosclerosis, presence of an indwelling venous catheter, use of oral contraceptives or androgens, immobility, morbid obesity). This drug is available in multiple products; the dose, indication, route of administration, method of preparation, requirements for reconstitution, and storage recommendations may vary (refer to specific product guidelines and verify that the correct product is chosen prior to administration). Children. Pregnancy and Lactation.
Adverse Reactions
Significant: Thromboembolic events (e.g. pulmonary embolism, MI, CVA, DVT), hypersensitivity reactions.
Gastrointestinal disorders: Nausea, vomiting, diarrhoea, dysgeusia, abdominal pain.
General disorders and administration site conditions: Fever, chills, inj site reactions.
Immune system disorders: Antibody development. Rarely, shock.
Musculoskeletal and connective tissue disorders: Joint swelling, arthralgia, myalgia.
Nervous system disorders: Headache, dizziness.
Respiratory, thoracic and mediastinal disorders: Nasopharyngitis, cough.
Skin and subcutaneous tissue disorders: Rash, pruritus, erythema.
Patient Counseling Information
Consider appropriate vaccination for patients with regular/repeated administration of this drug. To decrease the incidence of experiencing airway obstruction, seek immediate medical attention if this drug is self-administered to treat an acute laryngeal angioedema attack.
Monitoring Parameters
Monitor for signs and symptoms of hypersensitivity reactions (e.g. chest tightness, wheezing, hives, urticaria, hypotension, anaphylaxis) and thrombotic events.
Action
Description:
Mechanism of Action: C1-esterase inhibitor is a naturally occurring plasma glycoprotein that is a member of serine protease inhibitors (serpins) which has inhibiting potential on the complement system, intrinsic coagulation (contact system) pathway, fibrinolytic pathway, and the coagulation cascade. It forms complexes by binding to C1r and C1s, the active enzyme subunits of the first component of the complement system, hence blocking the classical pathway of the complement system. C1-esterase inhibitor is also the most important suppressor of contact system activation through inhibition of plasma kallikrein and factor XIIa which prevents the generation of bradykinin resulting in modulation of vascular permeability.
Onset: Symptom relief: Within 1 hour (50 international units/mL [IV]). Increased plasma C1-esterase inhibitor levels: Approx 1 hour or less (100 international units/mL [IV]).
Duration: 8.4 hours (50 international units/mL [IV]).
Pharmacokinetics:
Absorption: Slowly absorbed (500 international units/mL [SC]). Bioavailability: 43%. (500 international units/mL [SC]). Time to peak plasma concentration: Approx 4 hours (100 international units/mL [IV]); 59 hours (500 international units/mL [SC]).
Excretion: Elimination half-life: 22 hours; range: 17-24 hours (50 international units/mL [IV]). 56 hours; range 11-108 hours (100 international units/mL [IV]). 59 hours (500 International units/mL [SC]).
Storage
Intact vials: Store below 30°C. Do not freeze. Protect from light. Reconstituted solution: 50 International units/mL or 500 International units/mL: Store below 30°C for up to 8 hours; 100 International units/mL: Store between 15-25°C for up to 3 hours. Do not refrigerate or freeze. Storage recommendations may vary among countries and individual products (refer to specific product guidelines).
MIMS Class
Vaccines, Antisera & Immunologicals
References
Anon. C1 Inhibitor (Human). Lexicomp Online. Hudson, Ohio. Wolters Kluwer Clinical Drug Information, Inc. https://online.lexi.com. Accessed 03/01/2024.

Anon. C1-Esterase Inhibitor (Human). AHFS Clinical Drug Information [online]. Bethesda, MD. American Society of Health-System Pharmacists, Inc. https://www.ahfscdi.com. Accessed 03/01/2024.

Berinert (CSL Behring GmbH). DailyMed. Source: U.S. National Library of Medicine. https://dailymed.nlm.nih.gov/dailymed. Accessed 03/01/2024.

Berinert 3,000 International Units Powder and Solvent for Solution for Injection (CSL Behring GmbH). MHRA. https://products.mhra.gov.uk. Accessed 03/01/2024.

Berinert 500 International Units Powder and Solvent for Solution for Injection (CSL Behring GmbH). MIMS Hongkong. http://www.mims.com/hongkong. Accessed 03/01/2024.

Berinert 500 International Units Powder and Solvent for Solution for Injection/Infusion (CSL Behring GmbH). MHRA. https://products.mhra.gov.uk. Accessed 01/02/2024.

Cinryze 500 International Units Powder and Solvent for Solution for Injection (Takeda Manufacturing Austria AG). MHRA. https://products.mhra.gov.uk. Accessed 03/01/2024.

Cinryze Injection, Powder, Lyophilized, for Solution (Takeda Pharmaceuticals America, Inc.). DailyMed. Source: U.S. National Library of Medicine. https://dailymed.nlm.nih.gov/dailymed. Accessed 03/01/2024.

Haegarda Subcutaneous (CSL Behring GmbH). DailyMed. Source: U.S. National Library of Medicine. https://dailymed.nlm.nih.gov/dailymed. Accessed 01/02/2024.

Joint Formulary Committee. C1-Esterase Inhibitor. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 03/01/2024.

Paediatric Formulary Committee. C1-Esterase Inhibitor. BNF for Children [online]. London. BMJ Group, Pharmaceutical Press, and RCPCH Publications. https://www.medicinescomplete.com. Accessed 03/01/2024.

Disclaimer: This information is independently developed by MIMS based on C1-esterase inhibitor, human from various references and is provided for your reference only. Therapeutic uses, prescribing information and product availability may vary between countries. Please refer to MIMS Product Monographs for specific and locally approved prescribing information. Although great effort has been made to ensure content accuracy, MIMS shall not be held responsible or liable for any claims or damages arising from the use or misuse of the information contained herein, its contents or omissions, or otherwise. Copyright © 2024 MIMS. All rights reserved. Powered by MIMS.com
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