Hereditary angioedema managed with low-dose danazol and C1 esterase inhibitor concentrate: a case report.
BACKGROUND
Hereditary angioedema (HAE) is a rare life-threatening disease that can occur in pregnancy.


METHODS
A nulliparous woman was diagnosed as having HAE at 22 weeks of gestation after a series of symptomatic episodes. Following an initial course of C1 esterase inhibitor (C1EI) therapy for an acute episode of HAE, she was treated with danazol for prophylaxis. Danazol did not prevent recurrence of symptoms, its use was discontinued after six weeks. Thereafter, the patient was treated exclusively with C1EI at weekly intervals for exacerbations of her HAE. At 37 weeks' gestation, she delivered healthy 3050 g female neonate. At the time of discharge the female neonate had no signs of virilization or congenital anomalies.


CONCLUSIONS
Low dose danazol was ineffective in treating this woman's HAE in pregnancy. The use of C1EI in pregnancy is associated with good outcomes.
Department of Obstetrics and Gynaecology, Dalhousie University, Halifax NS.
Alon D
AD
Altman
Department of Obstetrics, St. Joseph's General Hospital, Comox BC.
Janice
J
McLaughlin
Division of Allergy and Immunology, Department of Medicine, University of British Columbia, Vancouver BC.
Robert
R
Schellenberg
Department of Internal Medicine, St. Joseph's General Hospital Comox BC.
Charles
C
Penner
Department of Medical Genetics, University of British Columbia Vancouver BC.
Laura
L
Arbour
Division of Hematology, Department of Medicine, University of British Columbia, Vancouver BC.
Peter
P
Tsang
Division of Hematology, Department of Medicine, University of British Columbia, Vancouver BC.
Penny
P
Ballem
Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynaecology, University of British Columbia, Vancouver BC.
Kenneth I
KI
Lim
D000328:Adult; D000799:Angioedema / Q000188:drug therapy* / Q000235:genetics; D003174:Complement C1 Inactivator Proteins / Q000627:therapeutic use*; D051056:Complement Inactivating Agents / Q000627:therapeutic use*; D003613:Danazol / Q000627:therapeutic use*; D004965:Estrogen Antagonists / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000188:drug therapy* / Q000235:genetics; D011256:Pregnancy Outcome; D012008:Recurrence
D003174:Complement C1 Inactivator Proteins; D051056:Complement Inactivating Agents; D004965:Estrogen Antagonists; D003613:Danazol