title: Lung structure and function similarities between primary ciliary dyskinesia and mild cystic fibrosis: a pilot study.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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. FEV1and 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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Translational Medical Sciences, Section of Pediatrics, Federico II University, Via Pansini 5, 80131, Naples, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Maglione
authors.affiliation: Department of Translational Medical Sciences, Section of Pediatrics, Federico II University, Via Pansini 5, 80131, Naples, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Montella
authors.affiliation: Biostructure and Bioimaging Institute, National Research Council, Naples, Italy.
authors.forename: Carmine
authors.initials: C
authors.lastname: Mollica
authors.affiliation: Department of Translational Medical Sciences, Adult Cystic Fibrosis Center, Federico II University, Naples, Italy.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Carnovale
authors.affiliation: Department of Translational Medical Sciences, Adult Cystic Fibrosis Center, Federico II University, Naples, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Iacotucci
authors.affiliation: Department of Translational Medical Sciences, Section of Pediatrics, Federico II University, Via Pansini 5, 80131, Naples, Italy.
authors.forename: Fabiola
authors.initials: F
authors.lastname: De Gregorio
authors.affiliation: Department of Translational Medical Sciences, Section of Pediatrics, Federico II University, Via Pansini 5, 80131, Naples, Italy.
authors.forename: Antonella
authors.initials: A
authors.lastname: Tosco
authors.affiliation: Department of Advanced Biomedical Sciences, Anatomo-Pathology Unit, Federico II University, Naples, Italy.
authors.forename: Mariarosaria
authors.initials: M
authors.lastname: Cervasio
authors.affiliation: Department of Translational Medical Sciences, Section of Pediatrics, Federico II University, Via Pansini 5, 80131, Naples, Italy.
authors.forename: Valeria
authors.initials: V
authors.lastname: Raia
authors.affiliation: Department of Translational Medical Sciences, Section of Pediatrics, Federico II University, Via Pansini 5, 80131, Naples, Italy. santamar@unina.it.
authors.forename: Francesca
authors.identifier: http://orcid.org/0000-0002-9375-7429
authors.initials: F
authors.lastname: Santamaria
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007619:Kartagener Syndrome / Q000175:diagnosis* / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D010865:Pilot Projects; D011446:Prospective Studies; D012720:Severity of Illness Index; D013147:Spirometry / Q000379:methods*; D013183:Sputum / Q000382:microbiology; D014057:Tomography, X-Ray Computed / Q000379:methods*; D055815:Young Adult
keywords: Computed tomography; Cystic fibrosis; Lung function; Magnetic resonance imaging; Primary ciliary dyskinesia