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Hereditary Angioedema (HAE)
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Hereditary angioedema (HAE) causes increases bradykinin causing angioedema, dry cough, abdominal pain, ...etc.
Bradykinin is produced from high molecular weight kininogen (HMWK) via plasma kallikrein.
High molecular weight kininogen is a plasma protein (alpha globulin) responsible for activating the intrinsic coagulation pathway as well as producing the inflammatory mediator known as bradykinin (via plasma kallikrein).
Plasma kallikrein, in addition to high molecular weight kininogen (HMWK), is responsible for activating factor XII into XIIa.
Kallikrein-Kinin system consists of a kallikrein (plasma kallikrein or tissure kallikrein) producing a kinin (bradykinin in the plasma and kallidin in the tissue).
Angiotensin-converting enzyme (ACE) degrades bradykinin into inactive metabolites.
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