소아, 임산부 및 수유부에서의 유전성 혈관부종: 전문가 의견서open accessManagement of hereditary angioedema in pediatric, pregnant, and breast-feeding patients: An expert opinion
- Other Titles
- Management of hereditary angioedema in pediatric, pregnant, and breast-feeding patients: An expert opinion
- Authors
- 윤선영; 정재우; 박소영; 김건우; 손경희; 강성윤; 박혜정; 강민규; 김주희; 박경희; 이동훈; 김세훈; 권혁수; 강혜련; 서동인
- Issue Date
- Jul-2022
- Publisher
- 대한 소아알레르기 호흡기학회
- Keywords
- Hereditary angioedema; C1-inhibitor; Child; Pregnant; Breast-feeding; .
- Citation
- Allergy Asthma & Respiratory Diseases, v.10, no.3, pp.131 - 138
- Journal Title
- Allergy Asthma & Respiratory Diseases
- Volume
- 10
- Number
- 3
- Start Page
- 131
- End Page
- 138
- URI
- https://scholarworks.bwise.kr/gachon/handle/2020.sw.gachon/85163
- DOI
- 10.4168/aard.2022.10.3.131
- ISSN
- 2288-0402
- 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.
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