Management of hereditary angioedema in pediatric, pregnant, and breast-feeding patients: An expert opinion
10.4168/aard.2022.10.3.131
- Author:
Sun Young YOON
1
;
Jae-Woo JUNG
;
So Young PARK
;
Gun-Woo KIM
;
Kyunghee SON
;
Sung-Yoon KANG
;
Hye Jung PARK
;
Min-Kyu KANG
;
Joo-Hee KIM
;
Kyung Hee PARK
;
Dong Hun LEE
;
Sae-Hoon KIM
;
Hyouk-Soo KWON
;
Hye-Ryun KANG
;
Dong In SUH
Author Information
1. Department of Internal Medicine, Chungnam National University Sejong Hospital, Sejong, Korea
- Publication Type:REVIEW
- From:Allergy, Asthma & Respiratory Disease
2022;10(3):131-138
- CountryRepublic of Korea
- Language:English
-
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.
