TY - JOUR
T1 - International guideline on the diagnosis and management of pediatric patients with hereditary angioedema
AU - Farkas, Henriette
AU - Martinez-Saguer, Inmaculada
AU - Bork, Konrad
AU - Germenis, Anastasios E.
AU - Grumach, Anete S.
AU - Horváth, Hanga Réka
AU - Luczay, Andrea
AU - Zanichelli, Andrea
AU - Magerl, Markus
AU - Betschel, Stephen
AU - Aygören-Pürsün, Emel
AU - Bernstein, Jonathan A.
AU - Boccon-Gibod, Isabelle
AU - Caballero, Teresa
AU - Cancian, Mauro
AU - Christiansen, Sandra
AU - Cohn, Danny M.
AU - Contreras, Francisco
AU - Craig, Sansanee
AU - Isaic, Camelia
AU - Jindal, Ankur
AU - Katelaris, Constance H.
AU - Longhurst, Hilary J.
AU - MacGinnitie, Andrew
AU - Peter, Jonny
AU - Porebski, Grzegorz
AU - Reshef, Avner
AU - Van Nguyen, Dinh
AU - Zuraw, Bruce
AU - Castaldo, Anthony J.
AU - Boysen, Henrik Balle
AU - Craig, Timothy
AU - Hereditary Angioedema Working Group,
PY - 2026
Y1 - 2026
N2 - Hereditary angioedema (HAE) with C1 inhibitor deficiency is a rare disease characterized by unpredictable episodes of tissue swelling (angioedema), which, in most cases, occur first under the age of 18 years, and entail a significant burden of disease not only for the patients but also for their families. Clinical symptoms of HAE are not specific, which may cause difficulties in differential diagnosis. Additionally, if not appropriately treated, HAE attacks can be life-threatening. The international HAE guidelines published so far have focused mainly on adults. A guideline that refers to the age-specific characteristics of pediatric patients, both in terms of diagnosis and management, was therefore needed. The International Steering Committee and Taskforce developed recommendations and provided evidence-based grading based on expert opinion and strength of evidence. Recommendations were presented to, discussed, and electronically voted by healthcare professionals during the 14th C1 Inhibitor Deficiency and Angioedema Workshop in Budapest, Hungary, 2025. This international guideline will ensure early diagnosis, standardized and up-to-date treatment, and promote the availability of effective therapies for all pediatric patients affected with this rare disease. It also draws attention to the importance of establishing HAE centers and registries, which solicit specialist care and research of the disease.
AB - Hereditary angioedema (HAE) with C1 inhibitor deficiency is a rare disease characterized by unpredictable episodes of tissue swelling (angioedema), which, in most cases, occur first under the age of 18 years, and entail a significant burden of disease not only for the patients but also for their families. Clinical symptoms of HAE are not specific, which may cause difficulties in differential diagnosis. Additionally, if not appropriately treated, HAE attacks can be life-threatening. The international HAE guidelines published so far have focused mainly on adults. A guideline that refers to the age-specific characteristics of pediatric patients, both in terms of diagnosis and management, was therefore needed. The International Steering Committee and Taskforce developed recommendations and provided evidence-based grading based on expert opinion and strength of evidence. Recommendations were presented to, discussed, and electronically voted by healthcare professionals during the 14th C1 Inhibitor Deficiency and Angioedema Workshop in Budapest, Hungary, 2025. This international guideline will ensure early diagnosis, standardized and up-to-date treatment, and promote the availability of effective therapies for all pediatric patients affected with this rare disease. It also draws attention to the importance of establishing HAE centers and registries, which solicit specialist care and research of the disease.
KW - acute treatment
KW - bradykinin
KW - C1 inhibitor deficiency
KW - complement test
KW - comprehensive care
KW - genetic testing
KW - hereditary angioedema
KW - long-term prophylaxis
KW - pediatric guideline
KW - short-term prophylaxis
UR - https://www.scopus.com/pages/publications/105030291603
U2 - 10.1111/all.70207
DO - 10.1111/all.70207
M3 - Article
C2 - 41618059
AN - SCOPUS:105030291603
SN - 0105-4538
JO - Allergy: European Journal of Allergy and Clinical Immunology
JF - Allergy: European Journal of Allergy and Clinical Immunology
ER -