Background Hereditary Angioedema (HAE) is a rare, inherited, autosomal dominant disease caused by a deficiency in C1-esterase inhibitor. It affects one in every 50,000 to 100,000 individuals. There were no approved treatments for HAE in North America until 2009, when C1-esterase inhibitor (Berinertâ) was released. It is an intravenous medication that requires patients to present to an emergency...

Background For a patient with Hereditary Angioedema (HAE), physiological and/or psychological stress can cause insufficient control of local inflammatory pathways. This leads to complement and contact system activation and excess bradykinin resulting in angioedema. Therefore, an invasive procedure or surgery can trigger an HAE attack; this in turn can cause further medical complications and pos...

A new form of hereditary angioedema (HAE) with normal C1 inhibitor (C1INH) was first described in 2000. The lack of clear diagnostic criteria, the heterogeneity among affected patients, and the varying names given to this disease have led to substantial confusion among both physicians and patients. This study was designed to bring more clarity to the diagnosis and potential treatment of HAE wi...

Hereditary angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency is characterized by recurrent attacks of swelling with symptoms typically beginning in childhood. The introduction of targeted therapies for prevention and treatment of attacks has been of significant benefit to patients, including children, with HAE. However, the ideal of a safe, convenient therapy that prevents all attacks ha...

Self-administration of therapy can help hereditary angioedema (HAE) patients regain control of their disease or reduce its impact and improve the quality of their lives. However, data from a self-administration survey, and subsequent discussion at an international HAE expert meeting, identified several barriers to self-administration therapy. These barriers include difficulty in administration ...

Propagation-based phase-contrast computed tomography (PPCT) utilizes highly sensitive phase-contrast technology applied to X-ray micro-tomography, especially with the extensive use of synchrotron radiation (SR). Performing phase retrieval (PR) on the acquired angular projections can enhance image contrast and enable quantitative imaging. We employed the combination of SR-PPCT and PR for the his...

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...

BACKGROUND Acute Renal Failure (ARF) in patients with cirrhosis is one of the most frequently encountered complications of obstructive jaundice. Marine organisms from the Mediterranean Coast of Egypt are considered potential sources of bioactive molecules. The present study was undertaken to explore the curative effects of Holothuria arenicola extract (HaE) against renal injury induced by bile ...

Hereditary angioedema (HAE) is a rare genetic condition that manifests as painful and potentially life-threatening episodic attacks of cutaneous and submucosal swelling. It results from functional deficiency of C1 esterase inhibitor (C1-INH), which is a regulator of the complement system, contact/kinin system and coagulation system. In HAE patients, the lowplasma concentration of functional C1-...

Hereditary Angiooedema (HAE) is characterized by a deficit or by a malfunctioning of C11nh; its symptoms vary greatly from one individual to another. Some studies suggest that some of the attacks of HAE may be triggered or affected by stress and emotional states (Zotter et al., 2014), however this link has never been examined in depth. Moreover, recent research has highlighted the influence of ...