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Early lung disease in young children with primary ciliary dyskinesia
Author(s) -
Brown David E.,
Pittman Jessica E.,
Leigh Margaret W.,
Fordham Lynn,
Davis Stephanie D.
Publication year - 2008
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.20792
Subject(s) - primary ciliary dyskinesia , medicine , disease , lung , lung disease , bronchiectasis , respiratory disease , bronchoscopy , kartagener syndrome , dyskinesia , pediatrics , cilium , pulmonary function testing , pathology , surgery , biology , parkinson's disease , microbiology and biotechnology
Primary ciliary dyskinesia (PCD) is an autosomal recessive disease in which ciliary dysfunction leads to chronic lung, sinus, and middle ear disease. PCD is often not diagnosed until late childhood due to its presumed rarity and the technical expertise necessary for diagnosis; as such, little is known about lung disease in young children with PCD. We report on 3 young children with PCD who had evidence of lung disease on infant pulmonary function testing, bronchoscopy, and/or computed tomography (CT) of the chest before 3 years of age. Pediatr Pulmonol. 2008; 43:514–516. © 2008 Wiley‐Liss, Inc.