[Hereditary angioedema. Control and treatment in 7 cases].
Hereditary angioedema (HAE) is due to a deficit of the C1 inhibitor (C1 INH) of a dominant autosomic inheritance. Seven patients are presented from a family with HAE, four of whom with poor prognosis due to the frequency and site of the angioedema. Prophylaxis was obtained with long-term danazol since antifibrinolytic drugs are not efficient in the prevention of outbreaks of angioedema. In three cases a concentrate of C1 INH was administered and in another as short term prophylaxis prior to surgery. C1 INH was more efficient under these indications than fresh plasma or antifibrinolytic drugs.
Marqués
L
L
Sección de Alergia, Hospital General Universitari Vall d'Hebron, Barcelona.
Dordal
T
T
Baltasar
M
M
Guspi
R
R
Nogueiras
C
C
Cadahia
A
A
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000799:Angioedema / Q000188:drug therapy* / Q000235:genetics; D003174:Complement C1 Inactivator Proteins / Q000627:therapeutic use*; D003181:Complement C4 / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree
D003174:Complement C1 Inactivator Proteins; D003181:Complement C4
Angioedema hereditario. Control y tratamiento en 7 casos.