Lung structure and function similarities between primary ciliary dyskinesia and mild cystic fibrosis: a pilot study(1926 views) Maglione M, Montella S, Mollica C, Carnovale V, Iacotucci P, De Gregorio F, Tosco A, Cervasio M, Raia V, Santamaria F
Department of Translational Medical Sciences, Section of Pediatrics, Federico II University, Via Pansini 5, Naples, 80131, Italy
Biostructure and Bioimaging Institute, National Research Council, Naples, Italy
Department of Advanced Biomedical Sciences, Anatomo-Pathology Unit, Federico II University, Naples, Italy
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Lung structure and function similarities between primary ciliary dyskinesia and mild cystic fibrosis: a pilot study
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. FEV
1
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).
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.
Lung structure and function similarities between primary ciliary dyskinesia and mild cystic fibrosis: a pilot study
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