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KMID : 1035620220100030131
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Allergy Asthma & Respiratory Disease
2022 Volume.10 No. 3 p.131 ~ p.138
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Management of hereditary angioedema in pediatric, pregnant, and breast-feeding patients: An expert opinion
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Yoon Sun-Young
Jung Jae-Woo
Park So-Young
Kim Gun-Woo
Son Kyung-Hee
Kang Sung-Yoon
Park Hye-Jung
Kang Min-Kyu
Kim Joo-Hee
Park Kyung-Hee
Lee Dong-Hun
Kim Sae-Hoon
Kwon Hyouk-Soo
Kang Hye-Ryun
Suh Dong-In
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Abstract
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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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KEYWORD
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Hereditary angioedema, C1-inhibitor, Child, Pregnant, Breast-feeding
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