Activation of kinin-kallikrein and complement pathways by oversulfated-chondroitin-sulfate (OSCS) has been linked with recent heparin-associated adverse clinical events. Given the fact that the majority of patients who received contaminated heparin did not experience an adverse event, it is of particular importance to determine the circumstances that increase the risk of a clinical reaction. In...

Methods We analyzed 137 edematous episodes requiring acute treatment and occurring in 6 C1-INH-HAE patients. The patients were treated at home with a dose of 2100 U rhC1-INH per occasion. They recorded the time of rhC1INH administration, time until the symptoms stopped worsening, time to the onset of symptom relief and to the complete resolution of symptoms. Any side effects were recorded in ad...

BACKGROUND For safe and efficacious treatment of hereditary angioedema, C1 esterase inhibitor (C1-INH) concentrates should have high purity and high amounts of functional protein. As no pharmacopoeia requirements exist for C1-INH concentrate lot release, biochemical characteristics as declared by the manufacturers may not be compared directly. This study compared the characteristics and purity ...

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...

Introduction Hereditary angioedema (HAE) is a rare, genetic disease with deficiency or the occurrence of defective C1 esterase inhibitor. The result acutely occurring subcutaneous submucosal edema in various parts body, which can range from cosmetically disruptive to extremely painful life-threatening. In addition physical consequences, unpredictability many attacks and subsequent restrictions ...