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 group

SUBMITTER: Maglione M 

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

REPOSITORIES: biostudies-literature