Characterization of acute hereditary angioedema attacks during pregnancy and breast-feeding and their treatment with C1 inhibitor concentrate.
OBJECTIVE
The objective of the study was to investigate the rates and characteristics of hereditary angioedema (HAE) attacks associated with pregnancy, delivery, and the postpartum period and their treatment with C1 esterase inhibitor (INH) concentrate.


METHODS
This was an observational study including 22 women with type I HAE, with data collected before, during, and after 35 pregnancies (37 children) based on patient diaries, interviews, and case report forms.


RESULTS
In 83% of pregnancies, attack rates increased during pregnancy; highest mean rates occurred in the second and third trimesters. C1-INH concentrate effectively controlled attacks and was safe for mothers and children. Low-plasma C1-INH activity during pregnancy tended to be associated with an increased chance of giving birth to a child with HAE.


CONCLUSIONS
Increased attack rates during pregnancy in women with HAE are well controlled with C1-INH concentrate, indicating the clear benefit of integrating the availability of C1-INH concentrate into the management plan for these women during pregnancy and delivery.
Martinez-Saguer
Inmaculada
I
Department of Hematology, Center of Pediatrics III, Johann Wolfgang Goethe University Hospital, Frankfurt am Main, Germany. inmaculada.martinez-saguer@kgu.de
Rusicke
Eva
E
Aygören-Pürsün
Emel
E
Heller
Christine
C
Klingebiel
Thomas
T
Kreuz
Wolfhart
W
D000208:Acute Disease; D054179:Angioedemas, Hereditary / Q000175:diagnosis / Q000188:drug therapy*; D001942:Breast Feeding; D015331:Cohort Studies; D050718:Complement C1 Inhibitor Protein / Q000008:administration & dosage*; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005260:Female; D005500:Follow-Up Studies; D030342:Genetic Diseases, Inborn / Q000175:diagnosis* / Q000453:epidemiology; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D015997:Neonatal Screening; D049590:Postpartum Period; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000175:diagnosis / Q000188:drug therapy*; D011256:Pregnancy Outcome; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome
D050718:Complement C1 Inhibitor Protein