Hereditary angioedema caused by C1-esterase inhibitor deficiency: a case report with literature-based analysis

Authors

  • Sufia Athar Department of Obstetrics and Gynecology, Al-Wakra Hospital, Hamad Medical Corporation, Al-Wakra, Qatar
  • Noureddine Korichi Department of Anaesthesiology, Al-Wakra Hospital, Hamad Medical Corporation, Al-Wakra, Qatar
  • Yousra Shehada Siam Department of Obstetrics and Gynecology, Al-Wakra Hospital, Hamad Medical Corporation, Al-Wakra, Qatar

DOI:

https://doi.org/10.18203/2320-1770.ijrcog20190314

Keywords:

C1-esterase inhibitor deficiency (HAE-C1-INH), CI inhibitor concentrate, Fresh frozen plasma (FFP), Hereditary angioedema (HAE), Plasma derived human C1 inhibitor concentrate (pdhC1INH), Pregnancy

Abstract

Hereditary angioedema (HAE) caused by C1-esterase inhibitor deficiency is an autosomal-dominant disease caused by a mutation in the C1-inhibitor gene. It is a rare disease that is often worsened during pregnancy and childbirth. HAE, though uncommon but if untreated it may lead to maternal death.  The case report presents the successful management of a 24 years old, G2P1, with hereditary angioedema caused by C1-esterase inhibitor deficiency. This patient was managed with a multidisciplinary approach by an obstetrician, an immunologist, an anaesthesiologist and a pediatrician. She had an uneventful antenatal period, labor was induced. She had precipitate delivery and soon after delivery had a flare up of the disease. It was successfully managed with fresh frozen plasma and close observation.

 

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Published

2019-01-25

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Section

Case Reports