Alon D Altman1, Janice McLaughlin2, Robert Schellenberg3, Charles Penner4, Laura Arbour5, Peter Tsang6, Penny Ballem6, Kenneth I Lim7. 1. Department of Obstetrics and Gynaecology, Dalhousie University, Halifax NS. 2. Department of Obstetrics, St. Joseph's General Hospital, Comox BC. 3. Division of Allergy and Immunology, Department of Medicine, University of British Columbia, Vancouver BC. 4. Department of Internal Medicine, St. Joseph's General Hospital Comox BC. 5. Department of Medical Genetics, University of British Columbia Vancouver BC. 6. Division of Hematology, Department of Medicine, University of British Columbia, Vancouver BC. 7. Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynaecology, University of British Columbia, Vancouver BC.
Abstract
BACKGROUND: Hereditary angioedema (HAE) is a rare life-threatening disease that can occur in pregnancy. CASE: 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. CONCLUSION: Low dose danazol was ineffective in treating this woman's HAE in pregnancy. The use of C1EI in pregnancy is associated with good outcomes.
BACKGROUND: Hereditary angioedema (HAE) is a rare life-threatening disease that can occur in pregnancy. CASE: 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. CONCLUSION: Low dose danazol was ineffective in treating this woman's HAE in pregnancy. The use of C1EI in pregnancy is associated with good outcomes.
Authors: Olivier Picone; Anne-Claire Donnadieu; François G Brivet; Catherine Boyer-Neumann; Véronique Frémeaux-Bacchi; René Frydman Journal: Obstet Gynecol Int Date: 2010-05-13