BACKGROUND Published literature documents the substantial burden of hereditary angioedema (HAE) with C1 inhibitor deficiency on the quality of life and work productivity of patients. However, despite advances in the field and the availability of guidelines to advise health care providers (HCP) on the diagnosis and management of HAE, there are still many challenges to overcome. For example, dela...

Hereditary angioedema (HAE) is a clinical disorder characterized by a deficiency of C1 esterase inhibitor (C1-INH). HAE has traditionally been divided into two subtypes. Unique among the inherited deficiencies of the complement system, HAE Types I and II are inherited as an autosomal dominant disorder. The generation of an HAE attack is caused by the depletion and/or consumption of C1-inhibitor...

BACKGROUND The diagnosis of hereditary angioedema (HAE) is often delayed due to the low awareness of this condition. In patients with undiagnosed HAE, abdominal symptoms often create the risk of unnecessary surgical operation and/or drug therapy. To explore the cause of misdiagnosis, we compared the laboratory findings of HAE patients under normal conditions with those during abdominal attacks....

Abscission is a developmental process that enables plants to shed unwanted organs. In Arabidopsis, the floral organ abscission is regulated by a signaling pathway consisting of the peptide ligand IDA, the receptor-like kinases (RLKs) HAE and HSL2, and a downstream MAP kinase (MAPK) cascade. However, little is known about the molecular link between ligand-receptor pairs and intracellular signali...

Objective: Sleeplessness is the most common sleep disorder. In this study the hypnotic effect of the hydro-alcoholic extract (HAE) of Perovskia abrotanoides and its water fraction (WF), ethyl acetate fraction (EAF) and n-butanol fraction (NBF) were studied in mice.Materials and Methods: The test compounds were administered intraperitoneally to mice 30 min before the administration of sodium pen...

Hereditary angioedema (HAE) is a rare disorder caused by the deficiency of the C1-inhibitor gene (C1INH) and characterized by recurrent bouts of angioedema. Autoimmune disorders frequently occur in HAE. Previously we found, that danazol has an adverse effect on serum lipid profile: reduced high-density lipoprotein (HDL) and elevated low-density lipoprotein (LDL) cholesterol levels are associate...

OBJECTIVE We tested the hypothesis that HDL-apolipoprotein A-I exchange (HAE), a measure of high-density lipoprotein (HDL) function and a key step in reverse cholesterol transport (RCT), is impaired in metabolic syndrome (MetSyn) patients who are asymptomatic for diabetes and cardiovascular disease. We also compared HAE with cell-based cholesterol efflux capacity (CEC) to address previous repor...

Dentists must take extreme care in treating patients with Hereditary Angioedema (HAE). Physical trauma, emotional stress, or anxiety during dental treatment could lead to an acute attack that may lead to a laryngeal obstruction. This article reviews clinical signs and symptoms, disease classification, pathophysiology, and treatment recommendations for HAE. Management of 2 patients with HAE is a...

BACKGROUND Hereditary angioedema (HAE) with normal C1 inhibitor (C1-INH) is a rare disorder. Mutations of the gene encoding coagulation factor XII have been identified in a subset of patients with this condition. Our aim was to investigate mutations in the F12 gene in patients with HAE with normal C1-INH from Brazil. METHODS We studied 5 Brazilian families with index female patients who prese...

Background Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare disease characterized by recurrent episodes of cutaneous, abdominal and laryngeal edema. Bradykinin is the mediator of increased vascular permeability and edema formation. Treatment of HAE attacks in Italy is based on the administration of human C1 inhibitor or bradykin receptor antagonist. A recombinant huma...