Allergy Asthma Respir Dis.  2022 Jul;10(3):131-138. 10.4168/aard.2022.10.3.131.

Management of hereditary angioedema in pediatric, pregnant, and breast-feeding patients: An expert opinion

Affiliations
  • 1Department of Internal Medicine, Chungnam National University Sejong Hospital, Sejong, Korea
  • 2Department of Internal Medicine, Chung-Ang University College of Medicine, Seoul, Korea
  • 3Department of Internal Medicine, St. Carollo General Hospital, Suncheon, Korea
  • 4Department of Internal Medicine, Kyung Hee University Medical Center, Seoul, Korea
  • 5Division of Pulmonology and Allergy, Department of Internal Medicine, Gil Medical Center, Gachon University College of Medicine, Incheon, Korea
  • 6Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
  • 7Departmemt of Internal Medicine, Chungbuk National University Hospital and Chungbuk National College of Medicine, Cheongju, Korea
  • 8Division of Pulmonary, Allergy, and Critical Care Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea
  • 9Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
  • 10Institute for Allergy, Yonsei University College of Medicine, Seoul, Korea
  • 11Department of Dermatology, Seoul National University College of Medicine, Seoul, Korea
  • 12Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
  • 13Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
  • 14Institute of Allergy and Clinical Immunology, Seoul National University Medical Research Center, Seoul, Korea
  • 15Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea
  • 16Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea

Abstract

Hereditary angioedema (HAE) is a rare inherited condition marked by recurrent skin and submucosal edema. HAE is caused by a C1 inhibitor deficiency or decreased C1 inhibitor function. The initial attack may occur during childhood or pregnancy, with symptoms ranging from classic angioedema to nonspecific stomach cramps. In this review, we discuss strategies for children and pregnant women to manage HAE attacks effectively and safely in light of the recent increase in HAE diagnosis. To begin, aggressive work-up is necessary to confirm HAE–1/2 and to determine the most effective countermeasures. Secondly, in the event of an acute attack, plasma-derived C1-inhibitor is the first line of defense for children and pregnant women. Icatibant is also appropriate for use, except in pregnant women. Fresh frozen plasma (FFP) may be suggested as an alternative. Thirdly, proactive measures to prevent HAE attacks should be considered whenever a procedure is performed that may result in an exacerbation. Finally, FFP, attenuated androgen and antifibrinolytic agents are recommended for long-term prophylaxis in South Korea where the C1-inhibitor is scarce. However, when making a decision, it is necessary to consider both the efficacy and the risk of adverse effects. For proper management, written action plans and first-aid kits are required. The action plans should be customized to the patients‘ unique circumstances.

Keyword

Hereditary angioedema; C1-inhibitor; Child; Pregnant; Breast-feeding
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