Search results for "Complement C1 Inhibitor Protein"
showing 5 items of 45 documents
Hereditary Angioedema: Increased Number of Attacks after Frequent Treatments with C1 Inhibitor Concentrate
2009
OBJECTIVE: C1 inhibitor concentrate is regarded as effective and safe in treating acute attacks of hereditary angioedema caused by C1 inhibitor deficiency. This study investigated the course of disease in 3 women treated frequently with C1 inhibitor concentrate. METHODS: Three women are described who received C1 inhibitor concentrate for the treatment of acute attacks of hereditary angioedema and experienced an increase in the frequency of attacks during that treatment period. In a control group of 24 patients aged more than 60 years with hereditary angioedema, the natural course of disease was determined. RESULTS: The 3 women (ages 50, 69, and 72 years) had received C1 inhibitor concentrat…
Hereditary angioedema: an update on causes, manifestations and treatment.
2019
Hereditary angioedema is a rare genetic disorder caused by deficiency of C1 esterase inhibitor (C1-INH) and characterized by recurrent episodes of severe swelling that affect the limbs, face, intestinal tract and airway. Since laryngeal oedema can be life-threatening as a result of asphyxiation, correct diagnosis and management of hereditary angioedema is vital. Hereditary angioedema attacks are mediated by bradykinin, the production of which is regulated by C1-INH. Hereditary angioedema therapy relies on treatment of acute attacks, and short- and long-term prophylaxis. Acute treatment options include C1-INH concentrate, icatibant and ecallantide. Self-administration of treatment is recomm…
Current status of implementation of self-administration training in various regions of Europe, Canada and the USA in the management of hereditary ang…
2013
Results from a 16-question survey about self-administration of hereditary angioedema (HAE) therapy, administered in Europe, Canada and the USA, were used to guide discussion at an international HAE expert meeting. The aim was to capture information about current practice in self-administered HAE therapy in these countries, including self-administration training, the key benefits of switching to self-administration, the barriers to self-administration and trends in self-administration. Overall, switching to self-administration therapy is looked upon favourably from both patient and clinician perspectives by virtue of the potential improvement in quality of life arising from optimisation of t…
Efficacy of Different Medical Therapies for the Treatment of Acute Laryngeal Attacks of Hereditary Angioedema due to C1-esterase Inhibitor Deficiency.
2016
Abstract Background Hereditary angioedema (HAE) is a rare disease characterized by C1-esterase inhibitor (C1-INH) deficiency, resulting in periodic attacks of acute edema, which can be life-threatening if they occur in the upper airway. No head-to-head comparisons of different treatment options for acute HAE attacks are available. Because immediate symptom relief is critical for potentially life-threatening laryngeal attacks, it is important to determine the treatment option that provides optimal treatment response. Objective Review and compare data from clinical studies that evaluated the efficacy and safety of treatments for laryngeal HAE attacks. Methods We conducted an indirect comparis…
The influence of the C1-inhibitor BERINERT® and the protein-free haemodialysate ACTIHAEMYL20%® on the evolution of the depth of scald burns in a porc…
1997
Standardized deep partial-thickness burns were inflicted on domestic pigs by scalding 30 per cent of the skin surface for 25 s with 75 degrees C hot water. The animals (n = 18; weight 25-35 kg) were divided into three groups: I, control group (n = 6), Ringer's lactate only; II, haemodialysate group (n = 6), Ringer's lactate and a protein-free haemodialysate of calf-blood (ACTIHAEMYL20%; AH) and III, C1-inhibitor group (n = 6), Ringer's lactate and C1-inhibitor (C1-INH; BERINERT). Skin biopsies were taken at defined time points (4, 28, 52 and 76 h) and investigated histologically. Depth of burn was determined morphometrically after coloration with a modified MTT-staining on frozen sections o…