Patients with hereditary angioedema (HAE) need a special concern during pregnancy. Although, the disease has a relatively benign course during pregnancy, maternal mortality has been reported. We present a HAE patient with recurrent attacks during pregnancy, but uncomplicated labor under C1INH concentrate prophylaxis.

Background Hereditary Angioedema (HAE) is an autosomal dominant disorder resulting from a deficiency of C1 esterase inhibitor (C1-INH). It is a rare disease with clinical manifestations debilitating and potentially fatal. The aim of this study was to report the clinical and laboratory characteristics and treatment of patients with Hereditary Angioedema with C1-INH deficit Outpatient Immunology ...

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...

Several studies suggest that the complement system is involved in atherogenesis. To further investigate this question, we have studied the ability of native and modified forms of LDL to bind and activate C1, the complex protease that triggers the classical pathway of complement. Unlike native LDL, oxidized (oxLDL) and enzymatically modified (E-LDL) derivatives were both recognized by the C1q su...

We present the successful application of C1 esterase inhibitor (C1INH) concentrate to a patient with clinical amniotic fluid embolism (AFE).

Hereditary angioedema (HAE) is a rare autosomal dominant disorder caused by reduced activity of the C1 esterase inhibitor. Clinically, angioedema most often involves the upper extremities, face, neck and larynx. The most common cause of death is asphyxia related to laryngeal oedema. Attacks are triggered by many factors such as trauma, stress, infections and hormonal fluctuations. C1 esterase i...

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...