Chiu Chun-Che, Wang Chao-Jan, Lee Wen-I, Wong Kin-Sun, Chiu Chih-Yung, Lai Shen-Hao
Division of Pediatric Pulmonology, Department of Pediatrics, Chang Gung Memorial Hospital at Linkou, and Chang Gung University, Taoyuan, Taiwan.
Department of Medical Imaging and Intervention, Chang Gung Memorial Hospital at Linkou, College of Medicine, Chang Gung University, Taoyuan, Taiwan.
J Microbiol Immunol Infect. 2020 Dec;53(6):1014-1020. doi: 10.1016/j.jmii.2020.01.006. Epub 2020 Feb 13.
Primary immunodeficiency (PID) accompanying with recurrent respiratory infections is thought to have a devastating effect on lung function. However, the associations between the airway structural abnormalities on chest computed tomography (CT), severity of dyspnea, and deterioration of pulmonary function test (PFT) have not been fully addressed.
Children diagnosed with PID in a tertiary referred center in northern Taiwan were enrolled. Demographic and clinical data including age, sex, age at diagnosis of PID, and follow-up period were collected. Chest CT images (modified Reiff scores), parameters of PFT, and life quality questionnaires (mMRC dyspnea scale) were analyzed and correlated using Spearman's rank correlation test.
A total of nineteen children with PID were enrolled and thirteen patients were diagnosed as having bronchiectasis based on chest CT scans. Modified Reiff scores of chest CT scan were negatively correlated with FEV (% predicted) and FEV/FVC ratio (P < 0.05). A strongly negative correlation was found between the mMRC dyspnea scale and FEV (% predicted) and FVC (% predicted), but positively correlated with RV (% predicted) and RV/TLC ratio (P < 0.05). Furthermore, there was a negative correlation between FVC (% predicted) with increasing follow-up period (P < 0.05).
In pediatric patients with PID, chest CT scan appears to be a good tool for not only the diagnosis of bronchiectasis, but also the degree of pulmonary function impairment. Further quality of life impairments could be particularly due to the airflow obstruction and air trapping related to bronchiectasis.
原发性免疫缺陷（PID）伴反复呼吸道感染被认为会对肺功能产生毁灭性影响。然而，胸部计算机断层扫描（CT）上的气道结构异常、呼吸困难严重程度和肺功能测试（PFT）恶化之间的关联尚未得到充分探讨。
纳入在台湾北部一家三级转诊中心诊断为PID的儿童。收集人口统计学和临床数据，包括年龄、性别、PID诊断年龄和随访期。使用Spearman等级相关检验分析并关联胸部CT图像（改良Reiff评分）、PFT参数和生活质量问卷（mMRC呼吸困难量表）。
共纳入19例PID患儿，13例患者根据胸部CT扫描诊断为支气管扩张。胸部CT扫描的改良Reiff评分与FEV（预测值%）和FEV/FVC比值呈负相关（P<0.05）。mMRC呼吸困难量表与FEV（预测值%）和FVC（预测值%）呈强负相关，但与RV（预测值%）和RV/TLC比值呈正相关（P<0.05）。此外，FVC（预测值%）与随访期延长呈负相关（P<0.05）。
在患有PID的儿科患者中，胸部CT扫描似乎不仅是诊断支气管扩张的良好工具，也是评估肺功能损害程度的良好工具。生活质量的进一步损害可能尤其归因于与支气管扩张相关的气流阻塞和气体潴留。
