Karalı Zuhal, Karalı Yasin, Çekiç Şükrü, Yazıcı Zeynep, Canıtez Yakup, Sapan Nihat, Gültekin Sara Şebnem Kılıç
Division of Pediatric Allergy and Immunology, Department of Pediatrics, Uludağ University, Faculty of Medicine, Bursa, Turkey.
Department of Radiology, Uludağ University, Faculty of Medicine, Bursa, Turkey.
Turk Pediatri Ars. 2020 Jun 19;55(2):174-183. doi: 10.14744/TurkPediatriArs.2020.46656. eCollection 2020.
To determine the frequency of sinopulmonary infections, detect changes in the respiratory system, and measure functional capacity of the lungs in our patients with humoral immunodeficiency.
Fifty-six patients with humoral immunodeficiency were enrolled in this study. The clinical, laboratory, and radiologic data, and pulmonary function tests of the subjects were evaluated from their file records, retrospectively.
The distribution of our patients was as follows: 25 patients had common variable immune deficiency, three patients had X-linked agammaglobulinemia, five patients had hyper immunoglobulin M syndrome, 19 patients had deficiency of immunoglobulin G subset, and four patients had selective immunoglobulin A deficiency. The most common symptom of the patients was chronic cough (n=47, 83.9%). The most common pathologies on high-resolution computed tomography of the chest were atelectasis and bronchiectasis (27.7%). The most common pathology in pulmonary function tests was the presence of moderate obstructive patterns along with restrictive patterns (n=6,12.5%). The FEV 1, FVC, and FEF 25-75 values were significantly lower in patients with common variable immunodeficiency compared with the patients who had IgG subset deficiencies (p=0.001, p=0.01, p=0.01). Among the patients who were treated with intravenous immunoglobulin, the age at the diagnosis of immunodeficiency was higher in patients with bronchiectasis (14.2±8.4 years) compared with those without bronchiectasis (10.1±11.4 years) (p=0.04).
Clinical findings are not sufficient to monitor the structural and functional changes in the respiratory system, and patients should be evaluated using high-resolution computed tomography of the chest and pulmonary function tests.
确定体液免疫缺陷患者中鼻窦肺部感染的频率,检测呼吸系统的变化,并测量其肺功能。
本研究纳入了56例体液免疫缺陷患者。回顾性地从他们的病历记录中评估受试者的临床、实验室和放射学数据以及肺功能测试结果。
我们患者的分布如下:25例患有常见变异型免疫缺陷,3例患有X连锁无丙种球蛋白血症,5例患有高免疫球蛋白M综合征,19例患有免疫球蛋白G亚群缺陷,4例患有选择性免疫球蛋白A缺陷。患者最常见的症状是慢性咳嗽(n = 47,83.9%)。胸部高分辨率计算机断层扫描最常见的病变是肺不张和支气管扩张(27.7%)。肺功能测试最常见的病变是中度阻塞性模式伴限制性模式(n = 6,12.5%)。与患有免疫球蛋白G亚群缺陷的患者相比,常见变异型免疫缺陷患者的第一秒用力呼气容积(FEV₁)、用力肺活量(FVC)和25%-75%用力呼气流量(FEF₂₅₋₇₅)值显著更低(p = 0.001,p = 0.01,p = 0.01)。在接受静脉注射免疫球蛋白治疗的患者中,患有支气管扩张的患者免疫缺陷诊断时的年龄(14.2±8.4岁)高于无支气管扩张的患者(10.1±11.4岁)(p = 0.04)。
临床发现不足以监测呼吸系统的结构和功能变化,应使用胸部高分辨率计算机断层扫描和肺功能测试对患者进行评估。