Background Hereditary Angioedema (HAE) is a disease caused by defective production or function of C1 inhibitor (C1INH) and transmitted by autossomic dominant inheritance pattern. Treatment of HAE is divided into three parts: short-term and long-term prophylaxis, and treatment of acute attacks. The long-term prophylaxis of HAE is aimed at reducing the frequency and severity of acute attacks. Thi...

BackgroundPhysician surveys on hereditary angioedema (HAE) management in 2010 and 2013 revealed important trends HAE care.ObjectiveTo evaluate current the impact of new treatment options physician practice patterns over time.MethodsDuring June July 2019, 5382 physicians were contacted by means postal mail to complete a 47-question survey; 177 responded (3%).ResultsAcross 3 surveys, home replace...

Kupffer cells (KC) represent the main part of the tissue macrophages. Beside phagocytosis of particulate material, involvement of KC in immunological and inflammatory reactions has been supposed. As C1 esterase inhibitor (C1-INH) is a serine protease inhibitor involved in such processes, the aim of this work was to study C1-INH synthesis in KC and, by comparison, in peritoneal macrophages (PM) ...

Hereditary angioedema (HEA) is a disease characterized by decreased levels or function of C1 esterase inhibitor (C1-INH). The symptoms of HEA in pediatric age group generally consist of recurrent episodes of soft tissue swelling. These symptoms can be transient, subtle, and varied in severity. Genitourinary system is rarely affected in this disease. Here, a three-year-old girl who presented wit...

C1 esterase inhibitor (Berinert®) is generally used to treat severe attack of hereditary angioedema. We describe here the case of a patient who presented with a severe angioedema induced by angiotensin-converting enzyme inhibitors (ACEIs) endangering her life. It could be successfully treated with that medicine.

BACKGROUND Complement activation during reperfusion of ischemic myocardium augments myocardial injury, and complement inhibition with C1-esterase inhibitor (C1-INH) at the time of reperfusion exerts marked cardioprotective effects in experimental studies. Application of C1-INH in newborns, however, was recently reported to have dangerous and even lethal side effects. This study addresses the es...

We present a new functional assay for the first complement component (C1) in plasma, based on its activation by inhibition of the C1-esterase inhibitor (C1-inh) when monospecific antiserum to C1-inh is added to the plasma. After maximal activation, we can determine the concentration of activated C1 by using an amidolytic rate assay with a chromogenic substrate. We have optimized the assay condi...

INTRODUCTION We report a rare, classic case of isolated angioedema of the bowel due to C1-esterase inhibitor deficiency. It is a rare presentation and very few cases have been reported worldwide. Angioedema has been classified into three categories. CASE PRESENTATION A 66-year-old Caucasian man presented with a ten-month history of episodic severe cramping abdominal pain, associated with loos...

INTRODUCTION Hereditary angioedema (HAE) is a genetic disease caused by C1-esterase inhibitor deficiency, characterized by recurrent attacks of intense, massive, localized subcutaneous oedema that can involve all parts of the body. The aim of this study is a comparison of the clinical effectiveness of conestat alfa, human C1 esterase inhibitor (C1INH), and icatibant in the treatment of acute an...

Hereditary angioedema secondary to C1-inhibitor deficiency is a rare autosomal dominant disorder characterized by a deficiency of C1 esterase inhibitor. An eight-year-old girl showed periorbital painless swelling, diagnosed as ethmoiditis. A craniofacial scan did not evidence a paranasal sinus involvement, C1INH levels were undetectable, with low C4 levels: 7.6 mg/dl and C1INH: <8.46 mg/dl. The...