Diagnosis and treatment of hereditary angioedema: An expert opinion

Jung, Jae-Woo; Park, Soyoung; Yoon, Sun Young; Kim, Gun-Woo; Sohn, Kyoung-Hee; Kang, Sung-Yoon; Park, Hye Jung; Kang, Min-Kyu; Kim, Joohee; Park, Kyung Hee; Suh, Dong In; Lee, Dong Hun; Kim, Sae‐Hoon; Kwon, Hyouk‐Soo; Kang, Hye Ryun

doi:10.4168/aard.2022.10.2.80

Cited by 3 publications

(1 citation statement)

References 52 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In response, healthcare professionals affiliated with the Korean Academy of Asthma, Allergy, and Clinical Immunology (KAAACI) who specialize in treating HAE have published expert opinions based on recent literature reviews and information [7] . This opinion paper aims to provide guidance for frontline clinicians, particularly regarding children, pregnant women, and breastfeeding mothers who require special consideration in managing HAE.…”

Section: Introductionmentioning

confidence: 99%

Management of Hereditary Angioedema in Pediatric, Pregnant, and Breast-Feeding Patients: An Expert Opinion – A Secondary Publication

Yoon,

Jung,

Park

et al. 2024

AOGR

View full text Add to dashboard Cite

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, an 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 patient’s unique circumstances.

show abstract

Section: Introductionmentioning

confidence: 99%