C1-inhibiting Factor
C1-inhibiting Factor Uses, Dosage, Side Effects, Food Interaction and all others data.
C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma. The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.
This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system. The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.
The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema. It has a long duration of action as it is given every 3-4 days prophylactically. Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.
| Trade Name | C1-inhibiting Factor |
| Generic | Human C1-esterase inhibitor |
| Human C1-esterase inhibitor Other Names | C1 Esterase Inhibitor (Human), C1 inhibitor, C1 inhibitor (human), C1 inhibitor human, C1-esterase inhibitor, human, C1-INH, C1-inhibiting factor, C1-inhibitor, plasma derived, Human C1 inhibitor, Human C1-esterase inhibitor, Plasma protease C1 inhibitor |
| Type | |
| Weight | 68000.0 Da |
| Protein binding | Data regarding the protein binding of the C1 esterase inhibitor is not readily available. |
| Groups | Approved |
| Therapeutic Class | |
| Manufacturer | |
| Available Country | |
| Last Updated: | September 19, 2023 at 7:00 am |
Uses
C1-inhibiting Factor is a C1 inhibitor used to prevent angioedema attacks associated with hereditary angioedema.
C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.
C1-inhibiting Factor is also used to associated treatment for these conditions: Acute attack of hereditary angioedema
How C1-inhibiting Factor works
The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them. Patients with hereditary angioedema have low levels of C1 esterase inhibitors. By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability. Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.
Toxicity
Data regarding overdoses in humans are not readily available. A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.
Food Interaction
No interactions found.Volume of Distribution
The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.
Elimination Route
A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU*h/mL. A 100 IU/kg dose reaches a Cmax of 2.3 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 10.6 ± 2.5 IU*h/mL.
Half Life
The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h. Subcutaneous administration produces a half life of 199.6 hours.
Clearance
The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.
Elimination Route
After nonspecific proteolysis, the amino acids from protein drugs are reused for protein synthesis or further broken down and eliminated by the kidneys.
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