青春期对C1抑制物缺乏所致遗传性血管性水肿发作的起始、频率、部位及严重程度的影响：来自意大利遗传性和获得性血管性水肿网络（ITACA）的一项调查。
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).
作者信息
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
机构信息
UOSD Allergologia, University Hospital of Padua, Padua, Italy.
Tor Vergata University Hospital, Rome, Italy.
出版信息
Front Pediatr. 2023 Apr 18;11:1141073. doi: 10.3389/fped.2023.1141073. eCollection 2023.
INTRODUCTION
Hereditary 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.
METHODS
Retrospective 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).
RESULTS
The proportion of symptomatic patients increased significantly after puberty (98.2% vs 83.9%, =0.002 in males; 96.3% vs 68,4%, <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, <0.001) and in males (1(1.92) vs 1.25(1.56) respectively, <0.001). The increase was greater in females. No significant differences were detected in attack location before and after puberty.
DISCUSSION
Overall, 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.
引言
C1抑制物缺乏所致遗传性血管性水肿受激素因素影响，女性患者的病程更为严重。我们的研究旨在深入探讨青春期对发作的起始、频率、部位及严重程度的影响。
方法
通过半结构化问卷收集回顾性数据，并由意大利遗传性和获得性血管性水肿网络（ITACA）的10个意大利参考中心提供。
结果
青春期后有症状患者的比例显著增加（男性为98.2%对83.9%，P = 0.002；女性为96.3%对68.4%，P < 0.001）；青春期后急性发作的月均值显著更高，女性（青春期前三年中位数（四分位间距）= 0.41（2），青春期后三年为2（2.17），P < 0.001）和男性（分别为1（1.92）对1.25（1.56），P < 0.001）均如此。女性的增加幅度更大。青春期前后发作部位未检测到显著差异。
讨论
总体而言，我们的研究证实了先前关于女性表型更严重的报道。青春期易导致血管性水肿发作次数增加，尤其是女性患者。
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