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Acquired C1 esterase inhibitor deficiency, also referred to as acquired angioedema (AAE), is a rare medical condition that presents as body swelling that can be life-threatening and manifests due to another underlying medical condition.
A C1-INH concentrate can be used for angio-oedema (C1-INH deficiency). [2] [3] Pneumococcus and Haemophilus infections can be prevented via immunization. [2] Epsilon-aminocaproic acid could be used to treat hereditary C1-INH deficiency, though the possible side effect of intravascular thrombosis should be weighed. [7]
C1-inhibitor (C1-inh, C1 esterase inhibitor) is a protease inhibitor belonging to the serpin superfamily. [5] Its main function is the inhibition of the complement system (C1r, C1s) to prevent spontaneous activation but also as the major regulator of the contact system (PK, FXIIa, and FXIa).
However, in hereditary angioedema with C1 inhibitor deficiency, C1 inhibitor is either reduced in quantity and function (type 1) or dysfunctional (type 2), this leads to bradykinin disinhibition and bradykinin mediated activation of bradykinin B1 receptor and bradykinin B2 receptor on endothelial cells (cells lining blood vessels). [7]
Lack of regulation of the classical complement pathway through the deficiency in C1-inhibitor results in episodic angioedema. [1] C1-inhibitor defiency can be hereditary or acquired, resulting in hereditary or acquired angioedema. [12] C1-inhibitor plays the role of inactivating C1r and C1s to prevent further downstream classical complement ...
An online survey conducted by the Cleveland Clinic of 1,174 men 18 years or older, found that 72% of men would rather do household tasks, such as cleaning the bathroom or mowing the lawn, than see ...
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