학술논문
소아, 임산부 및 수유부에서의 유전성 혈관부종: 전문가 의견서
Management of hereditary angioedema in pediatric, pregnant, and breast-feeding patients: An expert opinion
Management of hereditary angioedema in pediatric, pregnant, and breast-feeding patients: An expert opinion
Document Type
Article
Author
윤선영 / Sun Young Yoon; 정재우 / Jae-woo Jung; 박소영 / So Young Park; 김건우 / Gun-woo Kim; 손경희 / Kyunghee Son; 강성윤 / Sung-yoon Kang; 박혜정 / Hye Jung Park; 강민규 / Min-kyu Kang; 김주희 / Joo-hee Kim; 박경희 / Kyung Hee Park; 이동훈 / Dong Hun Lee; 김세훈 / Sae-hoon Kim; 권혁수 / Hyouk-soo Kwon; 강혜련 / Hye-ryun Kang; 서동인 / Dong In Suh
Source
Allergy asthma & respiratory disease. Jul 30, 2022 10(3):131
Subject
Language
Korean
ISSN
2288-0402
Abstract
1/2형 HAE는 드문 질환이나 진단이 지연될 경우 생활 및 삶의 질에 지대한 영향을 미치며 더 나아가 치명적인 결과를 야기할 수 있다. 한국은 서양에 비해 유병률이 낮으나 진단 자체의 어려움으로 인해 발굴되지 못한 환자가 있을 가능성이 높아 환자의 조기 진단 및 적절한 치료를 위한 일선의 노력이 중요하다. 이 의견서가 이러한 진단과 치료에 도움이 되기를 바라며, 더 나아가 현재 치료제 선택의 폭이 더 좁은 소아와 임산부, 수유부의 최적의 치료를 위한 필수 약제들의 국내 도입을 위한 노력이 필요하다.
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. (Allergy Asthma Respir Dis 2022;10:131-138)
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. (Allergy Asthma Respir Dis 2022;10:131-138)