The impact of puberty on the onset, frequency, location, and severity of attacks in hereditary angioedema due to C1-inhibitor deficiency: A survey from the Italian Network for Hereditary and Acquired Angioedema (ITACA)
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Published:2023-04-18
Issue:
Volume:11
Page:
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ISSN:2296-2360
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Container-title:Frontiers in Pediatrics
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language:
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Short-container-title:Front. Pediatr.
Author:
Cancian Mauro,Triggianese Paola,Modica Stella,Arcoleo Francesco,Bignardi Donatella,Brussino Luisa,Colangelo Caterina,Di Agosta Ester,Firinu Davide,Guarino Maria Domenica,Giardino Francesco,Giliberti Marica,Montinaro Vincenzo,Senter Riccardo
Abstract
IntroductionHereditary angioedema due to C1-inhibitor deficiency is influenced by hormonal factors, with a more severe course of disease in women. Our study aims to deepen the impact of puberty on onset, frequency, location and severity of attacks.MethodsRetrospective data were collected through a semi-structured questionnaire and shared by 10 Italian reference centers of the Italian Network for Hereditary and Acquired Angioedema (ITACA).ResultsThe proportion of symptomatic patients increased significantly after puberty (98.2% vs 83.9%, p=0.002 in males; 96.3% vs 68,4%, p<0.001 in females); the monthly mean of acute attacks was significantly higher after puberty, and this occurred both in females (median (IQR) = 0.41(2) in the three years before puberty vs 2(2.17) in the three years after, p<0.001) and in males (1(1.92) vs 1.25(1.56) respectively, p<0.001). The increase was greater in females. No significant differences were detected in attack location before and after puberty.DiscussionOverall, our study confirms previous reports on a more severe phenotype in the female gender. Puberty predisposes to increased numbers of angioedema attacks, in particular in female patients.
Publisher
Frontiers Media SA
Subject
Pediatrics, Perinatology and Child Health