过敏性疾病与遗传性血管性水肿的相关性分析

A. Aydoğdu, A. Aydoğdu
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摘要

遗传性血管性水肿(HAE)是一种罕见的常染色体显性遗传病,由C1酯酶抑制剂(C1- inh)缺乏或功能障碍引起。在有关遗传性血管性水肿相关疾病的研究中,与健康人群相比,过敏性疾病的发生率更高,但尚未开展相关研究。本研究是为了确定遗传性血管性水肿患者特应性敏感性和过敏性疾病共病的频率。研究纳入了2019年至2021年期间在梅尔辛市医院儿科过敏综合诊所诊断为遗传性血管性水肿的32例患者。在这些患者中,从他们的档案中获得有关特应性疾病存在的信息并进行记录。哮喘6例(18.8%),变应性鼻炎18例(46.9%),荨麻疹2例(6.2%),未见皮炎。Fx5检测阳性5例(15.6%),吸入性变应原混合物sps IgE检测阳性7例(25.9%)。IgE≥100者12例(52.2%),嗜酸性粒细胞≥4%者16例(50%)。总之,我们推测在本研究中,特应性疾病和特应性敏感性的关联在血管性水肿患者中比在健康人群中更常见。然而,需要更大样本的研究来证实这一发现。
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Analysis of the Association of Allergic Disease in Pediatric Patients with Hereditary Angioedema
Hereditary angioedema (HAE) is a rare autosomal dominant disease that is derived from the deficiency or dysfunction of C1 esterase inhibitor (C1-INH). In studies about the diseases that can accompany hereditary angioedema, allergic diseases occur more frequently compared to the healthy population but no studies on this issue have been carried out. This study was conducted in order to determine the frequency of the comorbidity of atopic sensitivity and allergic diseases in patients with hereditary angioedema. 32 patients who were diagnosed with hereditary angioedema in the Paediatric Allergy Polyclinic of Mersin City Hospital between 2019 and 2021 were included in the study. In these patients, the information about the existence of atopic diseases was obtained from their files and recorded. While asthma was observed in 6 (18.8%), allergic rhinitis in 18 (46.9%), and urticaria in 2 (6.2%) patients, dermatitis was not observed in any of the patients. While Fx5 test was positive in 5 (15.6%) patients, inhalant allergen mix sps IgE was positive in 7 (25.9%) patients. The IgE value was above 100 in 12 (52.2%) patients, and the eosinophil level was above 4% in 16 (50%) patients. In conclusion, we speculate in this study that the association of atopic disease and atopic sensitivity is observed more frequently in patients with angioedema than in the healthy population. However, a study with a larger sample is necessary to confirm this finding. 
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