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Hereditary Angioedema and Pregnancy
Abstract Number: S-49
Abstract Type: Case Report/Case Series
Introduction: Hereditary angioedema (HAE) is a rare autosomal-dominant disease produced by a deficiency of functioning C1 esterase inhibitor. The C1 esterase inhibitor is a plasma protease that blocks the activation of complement and the formation of bradykinin. Lack of this enzyme allows the complement cascade to proceed relatively unimpeded and results in clinical angioedema.1 Trauma, infection and stress are the most commonly reported triggers for HAE attacks. 2 Women with HAE have greatly reduced or absent attacks in the last two trimesters of pregnancy, although during the post-partum period, the majority of women with HAE experience increased frequency and severity of attacks.3 There is a paucity of information in the literature with respect to this disease and pregnancy. We present the management of a pregnant patient with HAE who delivered vaginally at full term.
Case presentation: Our patient is 32 year old G1 P 0 who presented to our hospital for delivery. The patient had a strong family history of HAE. Her brother, who received aggressive immunotherapy, had 10-15 episodes monthly, and both her mother and aunt had a tracheostomy during labor because of their angiodema. Our patient experienced 6-10 previous attacks, which resolved spontaneously without hospitalization. The patient did not experience any attacks during this pregnancy. After consultation with the obstetrical team the plan was to administer C1 esterase inhibitor if the patient developed any symptoms of angioedema. During her labor C1 esterase inhibitor and epinephrine were available in the room for immediate infusion. Tracheal intubation equipment was at the bedside to secure her airway if angiodema developed. When labor analgesia was requested a combined spinal-epidural anesthestic was performed using bupivacaine 2.5 mg for spinal injection followed by 0.125 % bupivacaine with 0.0002% fentanyl infusion through the epidural catheter. She vaginally delivered a male infant with APGAR scores of 9/9 without any complications. The patient remained hospitalized for four days and was then discharged home with no complications.
Discussion:HAE is an uncommon syndrome. Acute life threatening attacks can be treated by securing the airway and administering epinephrine, diphenhydramine, cimetidine and methyprednisolone. Chronic management can be achieved by corticosteroids, androgens, antihistamines and C1 esterase inhibitor infusions.4
1.Bowen T et al. Canadian 2003 international consensus algorithm for the diagnosis, therapy, and management of hereditary angioedema. J Allergy Clin Immunol. 2004; 114(3):629–37.
2.Frank MM et al. Hereditary angioedema: the clinical syndrome and its management. Ann Intern Med 1976, 84:580-593.
3.Chinniah N et al. Hereditary angioedema and pregnancy. Aust N Z J Obstet Gynaecol. 2009 Feb; 49(1):2-5.
4.Ferri: Ferri's Clinical Advisor 2012, 1st ed. http://www.mdconsult.com/books/page.do?eid=4-u1.0-B978-0-323-05611