[Hereditary angioedema].

Abstract

Hereditary angio-oedema (AOH) is a familial affliction that is most often hereditary, but sometimes acquired and linked to a deficiency of C1 esterase inhibitor. 8 cases have been examined, amongst which there was one attack in a young girl, without any clinical signs. Amongst the other 7, one had acquired AOH. The diagnosis was confirmed by the deficiency of C1 esterase inhibitor, associated with a significant reduction of the other fraction of complement c3-c4 and also total haemolytic complement. Strong doses of corticosteroids were used in 4 patients for urgent treatment. Basic treatment with DANAZOL was commenced in 3 patients.

• Authors: A Sabbah, I Marsollier, V Trouillard
• Journal: Allergie et immunologie (Allerg Immunol (Paris)) Vol. 21 Issue 8 Pg. 297-300 (Oct 1989) ISSN: 0397-9148 [Print] France
• Vernacular Title: L'angio-oedème héréditaire (AOH).
• PMID: 2803509 (Publication Type: English Abstract, Journal Article)

Chemical References

• Adrenal Cortex Hormones
• Complement C1 Inactivator Proteins
• Complement System Proteins
• Danazol

Topics

• Adolescent
• Adrenal Cortex Hormones (therapeutic use)
• Adult
• Angioedema (blood, drug therapy, genetics)
• Child
• Complement C1 Inactivator Proteins (deficiency, genetics)
• Complement System Proteins (deficiency)
• Danazol (therapeutic use)
• Female
• Humans
• Male