[Hereditary angioneurotic edema: an underestimated medical emergency. Apropos of 33 cases].
BACKGROUND
Hereditary angio-oedema is a poorly understood disease. In more than 70 p. 100 of the cases, diagnosis is made 5 to 10 years after the first manifestations despite their typical nature.


METHODS
We examined retrospectively 33 cases of hereditary angio-neurotic oedema treated at the Besançon University Hospital from 1973 to 1990.


RESULTS
Laryngeal oedema was the most dangerous complication causing a critical episode in 22 cases. Ten patients were given danazol.


CONCLUSIONS
This study emphasizes that preventive treatment is essential, based on danazol which should be prescribed at the lowest dose which gives a 50 p. 100 rise in the C2 complement fraction (usually 50 to 200 mg/day). Life-threatening attacks should be treated with purified C1 INH, although there are still problems with drug supply.
de Wazières
B
B
Service de Médecine interne et Immunologie clinique, Centre Hospitalier Universitaire, Besançon.
Dupond
J L
JL
Hory
B
B
Humbert
P
P
Wendling
D
D
Fest
T
T
D000293:Adolescent; D000328:Adult; D000799:Angioedema / Q000150:complications / Q000188:drug therapy / Q000235:genetics*; D003174:Complement C1 Inactivator Proteins / Q000627:therapeutic use; D003613:Danazol / Q000627:therapeutic use; D004630:Emergencies; D005260:Female; D006801:Humans; D007819:Laryngeal Edema / Q000209:etiology; D008297:Male; D012189:Retrospective Studies
D003174:Complement C1 Inactivator Proteins; D003613:Danazol
L'oedème angio-neurotique héréditaire: une urgence médicale sous-estimée. A propos de 33 cas.