Maglione Marco, Montella Silvia, Mollica Carmine, Carnovale Vincenzo, Iacotucci Paola, De Gregorio Fabiola, Tosco Antonella, Cervasio Mariarosaria, Raia Valeria, Santamaria Francesca
Department of Translational Medical Sciences, Section of Pediatrics, Federico II University, Via Pansini 5, 80131, Naples, Italy.
Biostructure and Bioimaging Institute, National Research Council, Naples, Italy.
Ital J Pediatr. 2017 Apr 12;43(1):34. doi: 10.1186/s13052-017-0351-2.
Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are increasingly compared. There are no chest magnetic resonance imaging (MRI) comparative studies of PCD and CF. We assessed clinical, functional, microbiological and MRI findings in PCD and mild CF patients in order to evaluate different expression of lung disease.
Twenty PCD (15.1 years) and 20 CF subjects with mild respiratory impairment (16 years, 70% with pancreatic insufficiency) underwent MRI, spirometry, and sputum cultures when clinically stable. MRI was scored using the modified Helbich system.
PCD was diagnosed later than CF (9.9 versus 0.6 years, p = 0.03), despite earlier symptoms (0.1 versus 0.6 years, p = 0.02). In the year preceding the study, patients from both groups underwent two systemic antibiotic courses (p = 0.48). MRI total scores were 11.6 ± 0.7 and 9.1 ± 1 in PCD and CF, respectively. FEV and FVC Z-scores were -1.75 (range, -4.6-0.7) and -0.6 (-3.9-1.8) in PCD, and -0.9 (range, -5.4-2.3) and -0.3 (-3.4-2.5) in CF, respectively. No difference was found between lung function or structure, despite a higher MRI subscore of collapse/consolidation in PCD versus CF (1.6 ± 0.1 and 0.6 ± 0.2, p < 0.001). These findings were confirmed after data-control for diagnostic delay. Pseudomonas aeruginosa and Staphylococcus aureus were more frequent in CF than in PCD (p = 0.05 and p = 0.003, respectively).
MRI is a valuable radiation-free tool for comparative PCD and CF lung disease assessment. Patients with PCD may exhibit similar MRI and lung function changes as CF subjects with mild pulmonary disease. Delay in PCD diagnosis is unlikely the only determinant of similarities.
原发性纤毛运动障碍（PCD）和囊性纤维化（CF）越来越多地被进行比较。目前尚无关于PCD和CF的胸部磁共振成像（MRI）对比研究。我们评估了PCD患者和轻度CF患者的临床、功能、微生物学及MRI表现，以评估肺部疾病的不同表现。
20例PCD患者（平均年龄15.1岁）和20例有轻度呼吸功能损害的CF患者（平均年龄16岁，70%有胰腺功能不全）在临床病情稳定时接受了MRI检查、肺功能测定和痰培养。MRI采用改良的赫尔比希系统进行评分。
尽管PCD患者症状出现更早（0.1年比0.6年，p = 0.02），但其诊断时间晚于CF患者（9.9年比0.6年，p = 0.03）。在研究前一年，两组患者均接受了两个疗程的全身抗生素治疗（p = 0.48）。PCD组和CF组的MRI总分分别为11.6±0.7和9.1±1。PCD组的第1秒用力呼气容积（FEV）和用力肺活量（FVC）Z评分分别为-1.75（范围：-4.6至0.7）和-0.6（范围：-3.9至1.8），CF组分别为-0.9（范围：-5.4至2.3）和-0.3（范围：-3.4至2.5）。尽管PCD组的肺不张/实变MRI分项评分高于CF组（1.6±0.1比0.6±0.2，p < 0.001），但两组在肺功能或结构方面未发现差异。在对诊断延迟进行数据控制后，这些结果得到了证实。铜绿假单胞菌和金黄色葡萄球菌在CF患者中比在PCD患者中更常见（分别为p = 0.05和p = 0.003）。
MRI是一种用于比较PCD和CF肺部疾病评估的有价值的无辐射工具。PCD患者可能表现出与轻度肺部疾病CF患者相似的MRI和肺功能变化。PCD诊断延迟不太可能是两者相似性的唯一决定因素。
