Dataset Information

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. FEV₁ 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?ConclusionsMRI 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.

SUBMITTER: Maglione M

PROVIDER: S-EPMC5389053 | biostudies-literature | 2017 Apr

REPOSITORIES: biostudies-literature

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Publications

Lung structure and function similarities between primary ciliary dyskinesia and mild cystic fibrosis: a pilot study.

Maglione Marco M Montella Silvia S Mollica Carmine C Carnovale Vincenzo V Iacotucci Paola P De Gregorio Fabiola F Tosco Antonella A Cervasio Mariarosaria M Raia Valeria V Santamaria Francesca F

Italian journal of pediatrics 20170412 1

<h4>Background</h4>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.<h4>Methods</h4>Twenty PCD (15.1 years) and 20 CF subjects with mild respiratory impairment (16 years, 70% with pancreatic insufficiency) underwent MRI, spi ...[more]

PMID: 28403885

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Dataset Information

Lung structure and function similarities between primary ciliary dyskinesia and mild cystic fibrosis: a pilot study.

Background

Methods

Results

Publications

Lung structure and function similarities between primary ciliary dyskinesia and mild cystic fibrosis: a pilot study.

Similar Datasets

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