Risk for Attacks in Hereditary Angioedema (HAE) Population Correlates with C1-inhibitor Functional Activity (C1-INHact)
نویسندگان
چکیده
منابع مشابه
Tryptase and histamine in patients with angioedema due to C1-inhibitor deficiency (Hereditary Angioedema, HAE) and in patients with mastocytosis
Material and methods. The determinations were performed in 14 mastocytotic patients, including 6 patients with the systemic mastocytosis and 8 with urticaria pigmentosa, in 14 with innate angio-motor oedema due to C1 inhibitor deficiency, and in 10 healthy controls. Tryptase levels were determined using PhadiaUniCAP TRYPTASE reagents. Histamine concentrations were measured using HISTAMINE ELISA...
متن کاملDiagnosis and treatment of hereditary angioedema with normal C1 inhibitor
Until recently it was assumed that hereditary angioedema is a disease that results exclusively from a genetic deficiency of the C1 inhibitor. In 2000, families with hereditary angioedema, normal C1 inhibitor activity and protein in plasma were described. Since then numerous patients and families with that condition have been reported. Most of the patients by far were women. In many of the affec...
متن کاملTreatment of hereditary angioedema with plasma-derived C1 inhibitor
BACKGROUND Plasma-derived C1 inhibitor (C1-INH) concentrate is a treatment option for acute hereditary angioedema (HAE) attacks and is considered the standard-of-care in many countries, although it is not yet available in the United States. Studies are still being conducted to establish its safety and efficacy as required by the FDA. OBJECTIVE To review the medical literature to determine if ...
متن کاملNeutrophil activation during attacks in patients with hereditary angioedema due to C1-inhibitor deficiency
BACKGROUND Earlier studies have shown that the absolute number of neutrophil granulocytes (NGs) may increase during attack of hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE). Whether NGs undergo activation during attack has not yet been investigated. However, as neutrophil elastase (NE) can cleave and inactivate C1-INH which may contribute to the dysregulation of the kallikrei...
متن کاملC1-inhibitor concentrate for treatment of hereditary angioedema.
BACKGROUND Hereditary angioedema due to C1 inhibitor deficiency is characterized by recurrent acute attacks of swelling that can be painful and sometimes life-threatening. METHODS We conducted two randomized trials to evaluate nanofiltered C1 inhibitor concentrate in the management of hereditary angioedema. The first study compared nanofiltered C1 inhibitor concentrate with placebo for treatm...
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ژورنال
عنوان ژورنال: Journal of Allergy and Clinical Immunology
سال: 2017
ISSN: 0091-6749
DOI: 10.1016/j.jaci.2016.12.750