Hereditary angioedema: diagnosis and management.
Hereditary angioedema is a rare autosomal dominant disorder due to the deficiency of functionally active C1-inhibitor. It is characterised by recurrent episodes of subcutaneous and mucosal edema. We report a case of hereditary angioedema presenting with the classic features of recurrent swelling of the extremities, abdominal pain and laryngeal edema. Serum complement C3 level was normal but C4 was low. She responded well to danazol and had no further attacks of angioedema.
Chng
H H
HH
Department of Medicine IV, Tan Tock Seng Hospital, Singapore.
Boey
M L
ML
D000328:Adult; D000799:Angioedema / Q000188:drug therapy / Q000235:genetics*; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D003613:Danazol / Q000627:therapeutic use; D005260:Female; D006801:Humans
D003174:Complement C1 Inactivator Proteins; D003613:Danazol