C1 Esterase Inhibitor Deficiency

C1 Esterase Inhibitor Deficiency. Hereditary angioedema commonly affects the limbs face intestinal tract and upper airway. This disorder can lead to.

The C1-INH test can also be used to check causes of unexplained inflammation or swelling known as edema and deficiency in C1 esterase inhibitor protein. The genetic defect is due to the heterozygous deficiency of C1-Inh that is transmitted as an autosomal dominant trait. N2 - Acquired C1 esterase inhibitor deficiency is a rare condition associated with autoimmune or low-grade lymphoproliferative disorders.

The precise incidence is unknown.

Hereditary angioedema otherwise known as C1 esterase deficiency is defined by recurrent episodes of angioedema without urticaria or pruritus. The complement system is a group of nearly 60 proteins in blood plasma or on the surface of some cells. C1 esterase inhibitor is a protein which is produced mainly in the liver and to some extent by activated Monocytes and other cell types. HAEGARDA C1 Esterase Inhibitor Subcutaneous Human is a plasma-derived concentrate of C1 Esterase Inhibitor C1-INH indicated for routine prophylaxis to prevent Hereditary Angioedema HAE attacks in patients 6 years of age and olderHAEGARDA is for subcutaneous use after reconstitution only.