Hereditary angioedema type II--a study of two families

Hereditary angioedema type II--a study of two families

Author Sole, D. Google Scholar
Leser, P. G. Google Scholar
Naspitz, C. K. Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Abstract Hereditary angioedema (HA) is caused by a quantitative or qualitative deficiency of C1 esterase inhibitor (C1 INH). We present a study of nine patients with HA belonging to two different families. The symptoms started before 10 years of age in most cases (78%). Facial edema (lips, eyes) and of the extremities (feet, hands) were the most frequent complaints. Three patients presented edema of the glottis and one of them underwent a tracheostomy twice. Laboratory tests, outside the acute crisis, revealed low levels of C4 in all patients. The serum levels of C1 INH were normal in seven patients; however, functional activity was not observed in any of them. After the use of a modified androgen (danazol), control of symptoms was observed in all patients, although functional activity was re-established in only five patients.
Keywords HEREDITARY ANGIOEDEMA
C1 ESTERASE INHIBITOR
COMPLEMENT DEFICIENCY
ANGIOEDEMA
Language English
Date 1992-11-01
Published in Journal Of Investigational Allergology & Clinical Immunology. Barcelona: J R Prous Sa, v. 2, n. 6, p. 318-322, 1992.
ISSN 1018-9068 (Sherpa/Romeo, impact factor)
Publisher J R Prous Sa
Extent 318-322
Access rights Closed access
Type Article
Web of Science ID WOS:A1992KL08000005
URI http://repositorio.unifesp.br/11600/44441

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