Background Hereditary angioedema (HAE) is a rare disorder characterized by a congenital deficiency of the C1-inhibitor. It is a potentially fatal illness as laryngeal edema may lead to asphyxiation. Treatment of HAE is mainly performed with C1-inhibitor concentrate, icatibant, attenuated androgens, and antifibrinolytic agents. We conducted a systematic review to evaluate the efficacy and safety...

somal dominant disorder that results in episodes of acute edema in various organs, including the gastrointestinal tract, skin and larynx. It is estimated to affect about 1 in 50 000–100 000 people. The symptoms, including abdominal pain, laryngeal edema and subcutaneous edema, usually begin in childhood and persist throughout life with unpredictable severity. Hereditary angioedema is classified...

Hereditary angioedema (HAE) is a potentially life-threatening disease caused by mutations in the gene encoding the serine protease inhibitor (serpin) C1 inhibitor (C1-inh). The mutations cause decreased functional plasma levels of C1-inh, which triggers unpredictable recurrent edema attacks. Subjects suffering from HAE have been classified in type I patients with decreased functional and antige...

THREE TYPES OF HEREDITARY ANGIOEDEMA (HAE) HAVE BEEN DESCRIBED two are due to C1 inhibitor (C1-INH) deficiency (C1-INH-HAE types I and II) and one is characterized by normal C1-INH (nC1-INH-HAE). The management of pregnancy in patients with HAE is often a clinical challenge owing to potential worsening of the disease in relation to the physiological increase in estrogens and the limited treatme...

The link among blood pressure, sympathetic output, and brain neurons producing catecholamines is well documented. Nevertheless, their intrinsic properties and any alterations in signaling characteristics between normotensive and hypertensive phenotypes remain unknown. Here, we directly compared neurophysiological properties of catecholamine release of C1 and A2 neurons of the spontaneously hype...

Bacteriophage P1 encodes several regulatory elements for the lytic or lysogenic response, which are located in the immC, immI, and immT regions. Their products are the C1 repressor of lytic functions with the C1 inactivator protein Coi, the C4 repressor of antirepressor synthesis and the modulator protein Bof, respectively. We have studied in vitro the interaction of the components of the immC ...

BACKGROUND Hereditary angioedema (HAE) due to C1 inhibitor deficiency manifests as recurrent swelling attacks that can be disabling and sometimes fatal. Long-term prophylaxis with twice-weekly intravenous injections of plasma-derived C1-inhibitor (pdC1-INH) has been established as an effective treatment. Subcutaneous (SC) administration of pdC1-INH has not been studied in patients with HAE. M...

OBJECTIVE To evaluate whether activated partial thromboplastin time (APTT) could be used in the laboratory diagnosis of hereditary or acquired angioedema (HAE or AAE) with and without C1 inhibitor (C1-INH) deficiency. METHODS In a prospective investigation, APTT and other coagulation parameters were determined in 149 adult patients with various types of angioedema and in 26 healthy participan...