Acute Hereditary Angioedema With Airway Compromise: Avoiding Airway Intervention With C1 Inhibitor Concentrate
نویسندگان
چکیده
منابع مشابه
C1-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...
متن کاملTreatment of 193 episodes of laryngeal edema with C1 inhibitor concentrate in patients with hereditary angioedema.
BACKGROUND Hereditary angioedema (HAE) is an autosomal dominant disease (Mendelian Inheritance in Man 106100) caused by an inherited deficiency of C1 inhibitor (C1-INH) function. The clinical symptoms include skin swelling, abdominal pain, and life-threatening episodes of upper airway obstruction. We evaluated the efficacy of C1-INH concentrate for treating sudden airway compromise. METHODS A...
متن کاملPhase II study results of a replacement therapy for hereditary angioedema with subcutaneous C1‐inhibitor concentrate
BACKGROUND Hereditary angioedema (HAE) due to C1 inhibitor deficiency manifests as recurrent swelling attacks that can be disabling and sometimes fatal. Long-term prophylaxis with twice-weekly intravenous injections of plasma-derived C1-inhibitor (pdC1-INH) has been established as an effective treatment. Subcutaneous (SC) administration of pdC1-INH has not been studied in patients with HAE. M...
متن کاملC1 esterase inhibitor deficiency, airway compromise, and anesthesia.
H ereditary angioedema (HAE) is a serious genetic abnormality involving the complement system and characterized by episodic and sometimes life-threatening airway edema. In 1882, von Quincke (1) published the first detailed description, and 3 yr later, Strubing used the term angioedema to describe this disorder. By 1888, Osler (2) had demonstrated the hereditary nature of the clinical presentati...
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...
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ژورنال
عنوان ژورنال: Immunology and Immunogenetics Insights
سال: 2018
ISSN: 1178-6345,1178-6345
DOI: 10.1177/1178634518786254