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►‍Antibiotics Lectures: https://www.medicosisperfectionalis.c... ... Check out my brand new "Electrolytes" course at https://www.medicosisperfectionalis.c... and use the PROMO code: ELECTROLYTES50 to get a 50% discount. Hereditary angioedema (HAE) is an autosomal dominant disorder characterized by a deficiency (or dysfunction) of the C1 esterase inhibitor (C1INH) leading to release of inhibition of plasma kallikrein which increases plasma bradykinin level.

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.

KallikreinKinin system consists of a kallikrein (plasma kallikrein or tissure kallikrein) producing a kinin (bradykinin in the plasma and kallidin in the tissue).

Angiotensinconverting enzyme (ACE) degrades bradykinin into inactive metabolites.

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In brief, hereditary angioedema (HAE) is an autosomal dominant disorder having a deficiency of C1 inhibitor (C1INH) causing increased bradykinin leading to angioedema, laryngeal edema, cough, abdominal pain, hypotension,...etc. ACE inhibitors are contraindicated. If you like my videos, please consider leaving a tip at https://www.paypal.me/perfectionalis/
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