Early lung disease in young children with primary ciliary dyskinesia

Pediatr Pulmonol. 2008 May;43(5):514-6. doi: 10.1002/ppul.20792.

Abstract

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.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Bronchitis / diagnosis
  • Bronchitis / drug therapy
  • Bronchitis / etiology
  • Bronchoscopy
  • Child, Preschool
  • Cilia / ultrastructure
  • Female
  • Humans
  • Infant
  • Kartagener Syndrome / complications*
  • Lung / diagnostic imaging
  • Lung Diseases / diagnosis
  • Lung Diseases / drug therapy
  • Lung Diseases / etiology*
  • Male
  • Microscopy, Electron
  • Otitis Media / diagnosis
  • Otitis Media / drug therapy
  • Otitis Media / etiology
  • Pneumonia, Pneumococcal / diagnosis
  • Pneumonia, Pneumococcal / drug therapy
  • Pneumonia, Pneumococcal / etiology
  • Pulmonary Atelectasis / diagnosis
  • Pulmonary Atelectasis / drug therapy
  • Pulmonary Atelectasis / etiology
  • Respiratory Function Tests
  • Sinusitis / diagnosis
  • Sinusitis / drug therapy
  • Sinusitis / etiology
  • Tomography, X-Ray Computed