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)
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 s...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2023-04-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2023.1141073/full |
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| author | Mauro Cancian Paola Triggianese Stella Modica Francesco Arcoleo Donatella Bignardi Luisa Brussino Caterina Colangelo Ester Di Agosta Davide Firinu Maria Domenica Guarino Francesco Giardino Marica Giliberti Vincenzo Montinaro Riccardo Senter |
| author_facet | Mauro Cancian Paola Triggianese Stella Modica Francesco Arcoleo Donatella Bignardi Luisa Brussino Caterina Colangelo Ester Di Agosta Davide Firinu Maria Domenica Guarino Francesco Giardino Marica Giliberti Vincenzo Montinaro Riccardo Senter |
| author_sort | Mauro Cancian |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-09T17:27:16Z |
| format | Article |
| id | doaj.art-952f860c14a44f08896c73d9c2d706ce |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-04-09T17:27:16Z |
| publishDate | 2023-04-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-952f860c14a44f08896c73d9c2d706ce2023-04-18T11:33:45ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602023-04-011110.3389/fped.2023.11410731141073The 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)Mauro Cancian0Paola Triggianese1Stella Modica2Francesco Arcoleo3Donatella Bignardi4Luisa Brussino5Caterina Colangelo6Ester Di Agosta7Davide Firinu8Maria Domenica Guarino9Francesco Giardino10Marica Giliberti11Vincenzo Montinaro12Riccardo Senter13UOSD Allergologia, University Hospital of Padua, Padua, ItalyTor Vergata University Hospital, Rome, ItalyTor Vergata University Hospital, Rome, ItalyUOC di Patologia Clinica e Immunologia, AOR Villa Sofia-Cervello, Palermo, ItalyIRCCS Ospedale Policlinico San Martino, Genova, ItalyUniversity Hospital Ordine Mauriziano di Torino, Turin, ItalyAzienda Sanitaria Locale di Pescara, Pescara, ItalyImmunoallergology unit, Careggi University Hospital, Florence, ItalyDepartment of Medical Sciences and Public Health, University of Cagliari, Cagliari, ItalyOspedale di Civitanova Marche, Civitanova Marche, Italy0Azienda Ospedaliero-Universitaria Policlinico “G.Rodolico-San Marco”, Catania, Italy1Azienda Ospedaliero-Universitaria Policlinico di Bari, Bari, Italy2Ospedale Regionale Generale F Miulli, Acquaviva delle Fonti, ItalyUOSD Allergologia, University Hospital of Padua, Padua, ItalyIntroductionHereditary 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.https://www.frontiersin.org/articles/10.3389/fped.2023.1141073/fullC1-inhibitor (C1INH)contact systemrare diseasehereditary angioedema (HAE)pediatricpuberty |
| spellingShingle | Mauro Cancian Paola Triggianese Stella Modica Francesco Arcoleo Donatella Bignardi Luisa Brussino Caterina Colangelo Ester Di Agosta Davide Firinu Maria Domenica Guarino Francesco Giardino Marica Giliberti Vincenzo Montinaro Riccardo Senter 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) Frontiers in Pediatrics C1-inhibitor (C1INH) contact system rare disease hereditary angioedema (HAE) pediatric puberty |
| title | 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) |
| title_full | 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) |
| title_fullStr | 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) |
| title_full_unstemmed | 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) |
| title_short | 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) |
| title_sort | 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 |
| topic | C1-inhibitor (C1INH) contact system rare disease hereditary angioedema (HAE) pediatric puberty |
| url | https://www.frontiersin.org/articles/10.3389/fped.2023.1141073/full |
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