Department of Diagnostic and Interventional Radiology.
Department of Diagnostic and Interventional Radiology with Nuclear Medicine, Thoraxklinik.
Ann Am Thorac Soc. 2024 Mar;21(3):438-448. doi: 10.1513/AnnalsATS.202305-453OC.
Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are characterized by inherited impaired mucociliary clearance leading to chronic progressive lung disease as well as chronic rhinosinusitis (CRS). The diseases share morphological and functional commonalities on magnetic resonance imaging (MRI) of the lungs and paranasal sinuses, but comparative MRI studies are lacking. To determine whether PCD shows different associations of pulmonary and paranasal sinus abnormalities on MRI and lung function test results in children (infants to adolescents) compared with children with CF. Eighteen children with PCD (median age, 9.5 [IQR, 3.4-12.7] yr; range, 0-18 yr) and 36 age-matched CF transmembrane conductance regulator modulator-naive children with CF (median age, 9.4 [3.4-13.2] yr; range, 0-18 yr) underwent same-session chest and paranasal sinus MRI as well as spirometry (to determine forced expiratory volume in 1 s percent predicted) and multiple-breath washout (to determine lung clearance index -score). Pulmonary and paranasal sinus abnormalities were assessed using previously validated chest MRI and CRS-MRI scoring systems. Mean chest MRI global score was similar in children with PCD and CF (15.0 [13.5-20.8] vs. 15.0 [9.0-15.0]; = 0.601). Consolidations were more prevalent and severe in children with PCD (56% vs. 25% and 1.0 [0.0-2.8] vs. 0.0 [0.0-0.3], respectively; < 0.05). The chest MRI global score correlated moderately with forced expiratory volume in 1 second percent predicted in children with PCD and children with CF ( = -0.523 and -0.687; < 0.01) and with lung clearance index in children with CF ( = 0.650; < 0.001) but not in PCD ( = 0.353; = 0.196). CRS-MRI sum score and mucopyocele subscore were lower in children with PCD than in children with CF (27.5 [26.3-32.0] vs. 37.0 [37.8-40.0] and 2.0 [0.0-2.0] vs. 7.5 [4.8-9.0], respectively; < 0.01). CRS-MRI sum score did not correlate with chest MRI score in PCD ( = 0.075-0.157; = 0.557-0.788) but correlated moderately with MRI morphology score in CF ( = 0.437; < 0.01). MRI detects differences in lung and paranasal sinus abnormalities between children with PCD and those with CF. Lung disease does not correlate with CRS in PCD but correlates in CF.
原发性纤毛运动障碍（PCD）和囊性纤维化（CF）的特征是遗传性黏液纤毛清除功能障碍，导致慢性进行性肺部疾病和慢性鼻旁窦炎（CRS）。这两种疾病在肺部和鼻旁窦的磁共振成像（MRI）上具有形态和功能的共同特征，但缺乏比较性 MRI 研究。本研究旨在确定与囊性纤维化相比，儿童（婴儿至青少年）的原发性纤毛运动障碍是否在 MRI 和肺功能测试结果上显示出不同的肺部和鼻旁窦异常关联。
18 名患有 PCD 的儿童（中位数年龄，9.5 [IQR，3.4-12.7] 岁；范围，0-18 岁）和 36 名年龄匹配的 CF 跨膜电导调节剂调节剂初治 CF 儿童（中位数年龄，9.4 [3.4-13.2] 岁；范围，0-18 岁）接受了胸部和鼻旁窦 MRI 以及肺活量测定（以确定 1 秒用力呼气量占预计值的百分比）和多次呼吸冲洗（以确定肺清除指数 -评分）的同期检查。使用先前验证的胸部 MRI 和 CRS-MRI 评分系统评估肺和鼻旁窦异常。PCD 和 CF 儿童的胸部 MRI 全球评分相似（15.0 [13.5-20.8] 与 15.0 [9.0-15.0]；= 0.601）。PCD 儿童的实变更为普遍且严重（56%与 25%和 1.0 [0.0-2.8] 与 0.0 [0.0-0.3]，分别； <0.05）。PCD 和 CF 儿童的胸部 MRI 全球评分与 1 秒用力呼气量占预计值百分比中度相关（= -0.523 和 -0.687；<0.01），与 CF 儿童的肺清除指数中度相关（= 0.650；<0.001），但与 PCD 儿童无关（= 0.353；= 0.196）。PCD 儿童的 CRS-MRI 总分和粘脓性窦分数低于 CF 儿童（27.5 [26.3-32.0] 与 37.0 [37.8-40.0] 和 2.0 [0.0-2.0] 与 7.5 [4.8-9.0]，分别；<0.01）。CRS-MRI 总分与 PCD 中胸部 MRI 评分无关（= 0.075-0.157；= 0.557-0.788），但与 CF 中 MRI 形态学评分中度相关（= 0.437；<0.01）。
MRI 检测到 PCD 和 CF 儿童之间肺部和鼻旁窦异常的差异。PCD 中的肺部疾病与 CRS 不相关，但在 CF 中与 CRS 相关。
