Glossary

| A-B | | C-E | | F-L | | M-R | | S-Z |
Angioedema (angioneurotic edema):
recurring episodes of non-inflammatory swelling.
Autosomal:
a chromosome other than a sex chromosome, normally occurring in pairs in somatic cells and singly in gametes.
Bradykinin:
a nonapeptide produced by activation of the kinin system in a variety of inflammatory conditions. It is a potent vasodilator and also increases vascular permeability, stimulates pain receptors, and causes contraction of a variety of extravascular smooth muscles.
C1-INH:
complement-1 inhibitor esterase, a serpin protease that inhibits numerous chemicals. C1-INH is the only inhibitor of C1r and C1s in the classical complement pathway. It also regulates kinin generation via inactivation of factors XIIa plasma and plasma kallikrein. In sum, C1-INH regulates the complement, contact, fibrinolytic, and coagulation cascades.
Endothelial:
pertaining to or made up of endothelium, which is the layer of cells that line the cavities of the heart, blood and lymph vessels, and the serous cavities of the body.
Epsilon-aminocaproic acid:
an epsilon amino acid that inhibits plasminogen activators and, to a lesser degree, plasmin.
Erythema marginatum:
occasionally seen in HAE, it is a non-raised rash that can be confused with serpiginous or annular hives.
Factor XIIa:
an activated form of coagulation factor XII. In the initial event in the intrinsic pathway of blood coagulation, kallikrein cleaves factor XII to XIIa. Factor XIIa is then further cleaved by kallikrein, plasmin, and trypsin to yield smaller factor XII fragments.
Fibrinolytic:
destroying or dissolving fibrillae, components of compound fibers.
Fibroblast:
a connective tissue cell.
Hepatocyte:
a hepatic cell.
Hereditary angioedema (HAE):
an autosomal dominant disease manifested by recurring episodes of edema of the skin or of the mucous membranes of the viscera and associated with either a deficiency of an esterase inhibitor in one of the components of the complement pathway or a functionally inactive form of the inhibitor.
Kallikrein:
any of several serine proteinases that cleave kininogens to form kinins, such as bradykinin or kallidin.
Kinin:
any of a group of vasoactive straight-chain polypeptides, formed by the kallikrein-catalyzed cleavage of kininogens, that cause vasodilation of most vessels but vasoconstriction of the pulmonary bed, and that also alter vascular permeability. Common examples are bradykinin and kallidin.
Kininogen:
either of 2 plasma α2-globulins that are kinin precursors, called high-molecular-weight K and low-molecular-weight K.
Laryngeal HAE attacks:
angioedema affecting or near the larynx (airway); laryngeal attacks can be fatal, and emergency tracheotomy is sometimes necessary to obtain a patent airway.
Megakaryocyte:
a giant cell 50 to 100 μm in diameter, with a greatly lobulated nucleus, found in the bone marrow; mature blood platelets are released from its cytoplasm.
Microglial:
of or pertaining to the microglia, or the small non-neural interstitial cells of mesodermal origin that form part of the supporting structure of the central nervous system. They are migratory and act as phagocytes to waste products of nerve tissue.
Monocyte:
a mononuclear phagocytic white blood cell; formed in the bone marrow and transported to tissues.
Pitting (pitting edema):
formation of well-defined, relatively deep depressions in a surface.
Prodromal:
symptoms that appear before an HAE attack.
Proteases:
any peptidase that catalyzes the cleavage of internal peptide bonds in a polypeptide or protein. They are divided into subclasses on the basis of catalytic mechanism. Also referred to as endopeptidases.
Pruritic:
of or characterized by pruritus, an unpleasant cutaneous sensation that provokes the desire to rub or scratch the skin.
Serpin (family):
any of a superfamily of high-molecular-weight, structurally related glycoproteins that act as serine protease inhibitors. Among their targets are serine endopeptidases involved in coagulation, complement activation, fibrinolysis, inflammation, and tissue remodeling.
Tranexamic acid:
an antifibrinolytic that acts by competitively inhibiting activation of plasminogen; it is used as a hemostatic in the prophylaxis and treatment of severe hemorrhage associated with excessive fibrinolysis, such as that following oral surgery in patients with hemophilia; administered orally or intravenously.
Urticaria:
skin condition characterized by welts that itch intensely, caused by an allergic reaction, an infection, or a nervous condition.

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Diagnosing HAE

HAE is often misdiagnosed because its symptoms mimic those of other conditions, such as allergic reaction, acute appendicitis, and gallbladder attack. Learn more about diagnosing HAE