title: [Hereditary and acquired angioedema: clinical characteristics in 8 patients and review of the literature].
abstract: C1 inhibitor disorders are a group of rare conditions in which the C1 inhibitor is deficient or defective. We present the clinical characteristics of 8 patients and a review of the literature. These are characterized by recurrent episodes of angioedema, which most often affect the skin or mucosal tissues of the upper respiratory and gastrointestinal tract. Laryngeal involvement may cause fatal asphyxiation. These disorders may be divided into two broad categories: hereditary angioedema (HAE) and acquired C1 inhibitor disorders. Indications for screening for HAE include: recurrent angioedema, episodic abdominal pain, laryngeal, a family background of angioedema, and a low C4 level. Acquired C1 inhibitor disorders are similar, but lack a family background. Treatment is divided into short and long-term prophylaxis with androgens, antifibrinolytics and C1 inhibitor replacement. First line therapy of acute attacks is C1 inhibitor.
authors.affiliation: Servicio de Medicina Interna, Hospital de Cabueñes, Gijón, Asturias, España. evamfonseca@yahoo.es
authors.forename: E M
authors.initials: EM
authors.lastname: Fonseca Aizpuru
authors.forename: E E
authors.initials: EE
authors.lastname: Rodríguez Avila
authors.forename: I
authors.initials: I
authors.lastname: Arias Miranda
authors.forename: F J
authors.initials: FJ
authors.lastname: Nuño Mateo
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000799:Angioedema / Q000175:diagnosis*; D054179:Angioedemas, Hereditary / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003174:Complement C1 Inactivator Proteins; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007819:Laryngeal Edema / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D012737:Sex Factors
chemical_list: D003174:Complement C1 Inactivator Proteins