Abstract
Objectives: Hereditary Angioedema (HAE) is a rare serious medical condition caused by a deficiency of C1-inhibitor, due to mutations in its structural gene. The disease appears clinically as cutaneous swelling of the extremities, face, genitals, and trunk, painful swelling of the gastrointestinal mucosa and life threatening laryngeal edema. In this study we evaluated in HAE patients and in the relative controls the psychological status and gender differences to verify if there is a link between disease and mental status. Methods: We studied “psychological stress”, using the Cognitive Behavioural Assessment 2.0 (CBA-2.0), in a total of 70 patients with confirmed HAE from different Italian Hospitals (41 women, 29 men; aged 17 to 78). Results: The analysis of the majority factors of CBA-2.0 tests indicates a clear difference between male and female patients; in fact, women perceive more intensively than men the different signs of the disease (have more consciousness of the consequences that this illness provokes in their life). Conclusion: Our data indicate that HAE is associated with emotional factors that can also complicate the clinical status of patients.
Highlights
Hereditary Angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency is a rare disease with an estimated prevalence of 1:50,000 in the general population
Angioedema attacks in HAE patients are characterized by activation of the systems controlled by C1-INH, i.e. contact, fibrinolytic, coagulation and complement that eventually leads to the release of bradykinin, the mediator of symptoms from High molecular weight Kininogen (HK) upon activation of the contact system, with the generation of activated factor XII (FXIIa) and plasma kallikrein from their precursor zymogens FXII and plasma prekallikrein [3,4,5,6,7,8]
Cognitive behavioural Assessment 2.0 includes an anamnestic schedule (Schedule 4) that consists of 59 items aimed to evaluate healthy conditions with specific emphasis on psychological problems that directly result from HAE [15]
Summary
Hereditary Angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency is a rare disease with an estimated prevalence of 1:50,000 in the general population. Angioedema attacks in HAE patients are characterized by activation of the systems controlled by C1-INH, i.e. contact, fibrinolytic, coagulation and complement that eventually leads to the release of bradykinin, the mediator of symptoms from High molecular weight Kininogen (HK) upon activation of the contact system, with the generation of activated factor XII (FXIIa) and plasma kallikrein from their precursor zymogens FXII and plasma prekallikrein [3,4,5,6,7,8] Both histamine and bradykinin bound specific receptors on endothelial cells H1 and BK-R2 respectively, which activate the nitric oxide pathway and contract endothelial cell junctions eventually allowing plasma to flow from the intravascular to the extravascular compartment to form the interstitial edema
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