Pediatric angioedema: Essential features and preliminary results from the Hereditary Angioedema Global Registry in Italy

被引:8
作者
Cancian, Mauro [1 ]
Perego, Francesca [2 ]
Senter, Riccardo [1 ]
Arcoleo, Francesco [3 ]
De Pasquale, Tiziana [4 ]
Zoli, Alessandra [5 ]
Cicardi, Marco [2 ,6 ]
Zanichelli, Andrea [7 ]
机构
[1] Univ Padua, Dept Med, Via Giustiniani 2, I-3526 Padua, Italy
[2] IRCCS Ist Clin Sci Maugeri, Milan, Italy
[3] Osped Riuniti Villa Sofia Cervello, Unita Operat Complessa Patol Clin, Palermo, Italy
[4] Civitanova Marche Hosp, Div Allergy, Civitanova Marche, Italy
[5] Osped Riuniti Ancona, Serv Immunol Clin & Tipizzaz Tessutale, Ancona, Italy
[6] Univ Milan, Dept Biomed & Clin Sci Luigi Sacco, Milan, Italy
[7] A5ST Fatebenefratelli Sacco, Milan, Italy
关键词
C1-inhibitor; disease registry; hereditary angioedema; HGR; ITACA; pediatric; DEFICIENCY; MANAGEMENT; CONSENSUS; DIAGNOSIS;
D O I
10.1111/pai.13170
中图分类号
R392 [医学免疫学];
学科分类号
100102 ;
摘要
Isolated angioedema, which is a localized, non-pitting, and transient swelling of the subcutaneous or submucosal tissue not associated with pruritus, urticaria, or anaphylaxis, may be classified, based on genetic pattern and mediators, respectively, as acquired or hereditary and histamine- or non-histamine-induced. The pediatric population with C1-INH-HAE (Hereditary angioedema due to C1-inhibitor deficiency) is mostly symptomatic. The frequency of symptoms in such a population compared to adults seems to be lower, but we need more prospective data to conclude on this point. The HGR (Hereditary angioedema global registry), which collects symptoms in real time, will probably provide such information. In terms of treatments, pediatric patients are significantly disadvantaged due to the few studies aimed at registering treatment for this population.
引用
收藏
页码:22 / 24
页数:3
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