What is C1 esterase inhibitor used for?

September 4, 2020 Off By idswater

What is C1 esterase inhibitor used for?

Complement C1 esterase inhibitor is used in people with hereditary angioedema. Berinert is used to treat attacks of angioedema. Cinryze and Haegarda are used to prevent attacks of angioedema.

What is the substrate for C1 esterase?

Although its name suggests specificity for C1, C1-INH acts on a variety of substrates including the contact system proteases (factor XII, plasma kallikrein), an intrinsic coagulation protease (factor XI) and the fibrinolytic proteases (plasmin, tissue plasminogen activator).

What is C1-inhibitor deficiency?

Background. Acquired angioedema due to C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is a serious condition that may result in life-threatening asphyxiation due to laryngeal edema. It is associated with malignant B-cell lymphoma and other disorders.

What is the treatment for hereditary angioedema?

Treatment of Hereditary Angioedema: Replacement therapy or immune modulating medicines pertaining to hereditary angioedema.
Generic Name Brand Name
Ecallantide Kalbitor Kalbitor Website
Icatibant Firazyr Firazyr Website
Kallikrein inhibitor- human

How do you test for low C1 inhibitors?

Your doctor can order blood tests to check your complement protein levels. One of these tests is the C1 esterase inhibitor test or C1-INH test. The C1-INH test can help your doctor determine if you have enough C1-INH.

What is C1 in blood test?

C1 esterase inhibitor (C1-INH) is a protein found in the fluid part of your blood. It controls a protein called C1, which is part of the complement system. The complement system is a group of nearly 60 proteins in blood plasma or on the surface of some cells.

What causes deficiency of C1 inhibitor?

The main causes of acquired C1-inhibitor deficiency are lymphoproliferative disease and autoimmune disease.

How do you treat hereditary angioedema?

Medicines called danazol and oxandrolone can help reduce the chances of swelling occurring if you have hereditary angioedema. These medicines boost the levels of C1 esterase inhibitor in your blood. Low levels of this substance are what causes the swelling.

How do you infuse HAEGARDA?

Draw air into an empty, sterile syringe. Use a silicone-free syringe. While the HAEGARDA vial is upright, screw the syringe to the Mix2Vial transfer set. Inject air into the HAEGARDA vial.