Journal
IMMUNOLOGY AND ALLERGY CLINICS OF NORTH AMERICA
Volume 26, Issue 4, Pages 709-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.iac.2006.09.003
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Until recently it was assumed that hereditary angioedema is a disease that results exclusively from a deficiency of the C1 inhibitor. In 2000, families with hereditary angioedema, normal C1 inhibitor activity, and protein in plasma were described; all patients were women. In many of the affected women, oral contraceptives, hormone replacement therapy containing estrogens, and pregnancies triggered the clinical symptoms. Recently, in some families mutations in the coagulation factor XII (Hageman factor) gene were detected in the affected women.
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