The diagnosis of C1-INH-HAE was established based on complement testing (complement C4 and C1 inhibitor
antigenic levels) and characteristic clinical features such as upper airway and subcutaneous swelling or abdominal pain (14).
International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor
A nationwide survey of hereditary angioedema due to C1 inhibitor
deficiency in Italy.
Barnstedt, "Treatment of 193 episodes of laryngeal edema with C1 inhibitor
concentrate in patients with hereditary angioedema," JAMA Internal Medicine, vol.
Bouillet et al., "International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor
deficiency," The Journal of Allergy and Clinical Immunology, vol.
While functional anti-FceRIa and anti-lgE have the potential to play a role in histamine-mediated angioedema, C1 inhibitor
(INH) deficiency or dysfunction, sometimes due to anti-C1 INH, plays a role in bradykinin (BK)-mediated angioedema.
Hereditary angioedema due to C1 inhibitor
deficiency: Patient registry and approach to the prevalence in Spain.
All tested patients had normal complement C1 inhibitor
levels and function.
There are several risk factors that can contribute to ACE inhibitor-induced angioedema, including previous angioedema, age above 65, NSAID use, female sex, smoking, seasonal allergies, certain immunosuppressants (sirolimus and everolimus), underlying C1 inhibitor
deficiency or dysfunction, history of ACE inhibitor-induced cough, and surgery [8-10].
However, lowered C1 inhibitor
function and normal quantitative C1 inhibitor
, C4, C1q and C3 complement components values were recorded in one patient.
Angioedema can occur in patients with decrease serum levels or abnormal function of regulatory complement protein, C1 inhibitor
Small bowel angioedema due to acquired C1 inhibitor
deficiency: a case report and overview.
Finally, in one article the potential role of MBL (mannose-binding lectin, a pattern recognition protein of the lectin pathway of complement) in the pathogenesis of human CIN and the beneficial effects we may obtain in clinical practice by its inhibition with the C1 inhibitor
, a potent MBL and lectin pathway inhibitor, are reviewed.