C1-esterase inhibitor

C1-esterase inhibitor

(see won ess-ter-aze in-hib-it-or) ,


(trade name)


Therapeutic: none assigned
Pregnancy Category: C


Treatment of acute abdominal, laryngeal, or facial attacks of hereditary angioedema (HAE).


Replaces malfunctioning or missing C1 esterase inhibitor in patients with HAE. Suppression of the contact activation system by C1-esterase inihibor prevents the cascade of events that leads to attacks of angioedema in HAE that are marked by increased vascular permeability, swelling, edema and laryngospasm.

Therapeutic effects

Lessened severity of HAE.


Absorption: IV administration results in complete bioavailability.
Distribution: Unknown.
Metabolism and Excretion: Unknown.
Half-life: 18 hr.

Time/action profile (symptom relief)

IVwithin 30 min1.5 hr>4 hr


Contraindicated in: History of life-threatening immediate hypersensitivity reactions, including anaphylaxis to C1 esterase inhibitor preparations.
Use Cautiously in: Risk factors for thrombotic events; Obstetric / Lactation: Use during pregnancy/lactation only if clearly needed; Pediatric: Children <12 yr (safety and effectiveness not established).

Adverse Reactions/Side Effects

Central nervous system

  • headache (most frequent)


  • thromboembolic events (life-threatening)


  • abdominal pain (most frequent)
  • diarrhea (most frequent)
  • nausea (most frequent)
  • vomiting (most frequent)
  • abnormal taste


  • muscle spasms (most frequent)


  • hypersensitivity reactions including ANAPHYLAXIS and EXACERBATION OF HAE (life-threatening)
  • ↑ severity of pain from HAE


Drug-Drug interaction

None noted.


Intravenous (Adults) 20 units/kg.


Lyophilized powder for intravenous use (requires reconstitution): 500 units/vial

Nursing implications

Nursing assessment

  • Assess for signs (facial, laryngeal, or abdominal swelling, dyspnea, pain, nausea, vomiting, cramps, diarrhea) and frequency of HAE.
  • Monitor for signs and symptoms of severe hypersensitivity reactions (hives, urticaria, chest tightness, wheezing, hypotension, anaphylaxis) during or after injection.
  • May cause thrombosis if used in doses exceeding 20 units/kg.

Potential Nursing Diagnoses

Activity intolerance


  • Intravenous Administration
  • Intermittent Infusion: Diluent: Medication and diluent vials should be at room temperature prior to dilution. Use diluent provided by manufacturer (10 mL sterile water) and transfer using the Mix2Vial transfer set and following manufacturer's guidelines. Gently swirl vial to ensure medication is fully dissolved; do not shake. Solution should be clear and colorless; do not administer solutions that are cloudy, discolored or contain a precipitate. Reconstituted solution is stable for 8 hrs; do not refrigerate or freeze. Do not use solutions beyond expiration date. If patient is to receive more than 1 vial, the contents of multiple vials may be pooled in a single administration syringe.
  • Rate: Administer via slow IV injection at a rate of 4 mL/min.
  • Y-Site Incompatibility: Do not mix with other products and administer via a separate infusion line.

Patient/Family Teaching

  • Explain purpose of medication to patient. Advise patient to read Patient Package Insert.
  • May be self-administered with proper training. Patient must be able to recognize the signs and symptoms of HAE and must have the dexterity and comprehension to self-administer injection. Instruct patient using directions in Patient Package Insert. Advise patient to seek medical attention immediately in addition to injection due to potential. for laryngeal edema. Instruct patient to record lot number from vial. At first sign of HAE attack, prepare injection. Bring C1-esterase inhibitor with you to health care facility. Notify health care professional immediately if swelling does not decrease after injection.
  • Inform patient that this medication is made from human blood and may carry a risk of transmitting infectious agents (viruses and possibly Creutzfeldt-Jakob [CJD]). Report all infections to the CSL Behring Pharmacovigilance Department at 1-866-915-6958.
  • Advise patient to notify health care professional immediately of signs of hypersensitivity reactions or thrombosis (new onset of swelling and pain in limbs or abdomen, new onset of chest pain, shortness of breath, loss of sensation or motor power, altered consciousness, vision, or speech) occur.
  • Caution patient to consult health care professional of planning to travel.
  • Advise female patients to notify health care professional if pregnancy is planned or suspected or if breastfeeding.

Evaluation/Desired Outcomes

  • Decrease in intensity of symptoms of HAE.
References in periodicals archive ?
PHILADELPHIA, March 18 /PRNewswire/ -- C1-esterase inhibitor (C1-INH) concentrate is a safe and effective therapy that rapidly relieves acute abdominal and facial swelling attacks in patients with hereditary angioedema (HAE), a rare and serious genetic disorder, according to data presented today at the American Academy of Allergy, Asthma & Immunology (AAAAI) 64th Annual Meeting.
During the past ten months, Lev has been building its commercial team and is now poised to complete launch preparations, including building a sales force, to support the commercialization of its lead product candidate, C1-esterase inhibitor (C1-INH), to be marketed as Cinryze(TM), for the treatment of hereditary angioedema (HAE), if and when approved by the FDA.
Phase III trial of C1-esterase inhibitor (C1-INH) for the acute treatment of hereditary angioedema (HAE).
Company Updates Development Timeline for C1-Esterase Inhibitor Replacement Therapy
Sinai and a patient with HAE both spoke about Lev's C1-esterase inhibitor (C1-INH), which is in Phase III trials and has been used in Europe for over 30 years.
RUCONEST, the first and only recombinant (non-blood-derived) C1-esterase inhibitor replacement therapy, was first approved by the EMA in June 2010 for the treatment of acute attacks of Hereditary Angioedema ("HAE").
Food and Drug Administration yesterday approved Ruconest, the first recombinant C1-Esterase Inhibitor product for the treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE).
20, 2014 /PRNewswire/ -- CSL Behring today announced it has enrolled the first patient in COMPACT , an international phase III study of a volume-reduced, subcutaneous formulation of C1-esterase inhibitor (C1-INH) concentrate in patients with frequent hereditary angioedema (HAE) attacks (NCT01912456).
Lev's product candidates are based on C1-esterase inhibitor ("C1-INH"), a human plasma protein that mediates inflammation and is potentially applicable as a treatment for a range of medical indications.
MARBURG, Germany, April 16, 2013 /PRNewswire/ -- CSL Behring today announced that European health authorities have approved an extended use of Berinert , a C1-esterase inhibitor (C1-INH) concentrate, for pre-procedure prevention (short-term prophylaxis) of acute episodes of hereditary angioedema (HAE), in adult and pediatric patients undergoing medical, dental or surgical procedures.
Subjects will receive ascending doses of mutant prourokinase or placebo, preceded, or not, by a bolus of a specific inhibitor called C1-esterase inhibitor or a C1-esterase inhibitor-placebo.
The paper entitled Nanofiltered C1-Esterase Inhibitor for The Acute Management and Prevention of Hereditary Angioedema Attacks Due to C1-Inhibitor Deficiency in Children by Dr.