title: Primary ciliary dyskinesia in six patients with bronchiectasis.
abstract: BACKGROUND
  Primary ciliary dyskinesia [PCD] is generally considered as a rare autosomal recessive disorder. Previous studies reported various prevalence of PCD among patients with bronchiectasis.
  
  
  METHODS
  Six PCD patients who were diagnosed during the investigation of 40 patients with bronchiectasis were enrolled in this study. Ultra structural studies for both epithelium and cilia were performed, and the deformities in detailed electron microscopic images confirmed the diagnosis of PCD.
  
  
  RESULTS
  Four patients experienced the first symptoms shortly after the birth, 1 by the age of 1 and 1 by the age of 4 years. Except of 1 case that was diagnosed 2 months after the onset of disease, diagnosis delay was longer than 5 years in all patients. Consanguineous marriage was observed in the parents of all patients. Upper respiratory tract infections were documented for all patients.
  
  
  CONCLUSIONS
  PCD should be considered as a probable underlying disorder in patients with bronchiectasis. Past medical history of otitis media and history of similar clinical findings in family members should raise suspicion toward PCD.
authors.forename: Mohammadreza
authors.initials: M
authors.lastname: Modaresi
authors.forename: Bamdad
authors.initials: B
authors.lastname: Sadeghi
authors.forename: Payam
authors.initials: P
authors.lastname: Mohammadinejad
authors.forename: Sayed Javad
authors.initials: SJ
authors.lastname: Sayedi
authors.forename: Farzad
authors.initials: F
authors.lastname: Masiha
authors.forename: Rohola
authors.initials: R
authors.lastname: Shirzadi
authors.forename: Gholamreza
authors.initials: G
authors.lastname: Azizi
authors.affiliation: Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. aghamohammadi@tums.ac.ir.
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
mesh_terms: D000293:Adolescent; D000328:Adult; D001987:Bronchiectasis / Q000150:complications* / Q000175:diagnosis*; D002648:Child; D002923:Cilia / Q000473:pathology* / Q000648:ultrastructure; D003241:Consanguinity; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007619:Kartagener Syndrome / Q000175:diagnosis*; D008297:Male; D008854:Microscopy, Electron; D010033:Otitis Media / Q000150:complications; D020545:Respiratory Mucosa / Q000648:ultrastructure; D012141:Respiratory Tract Infections / Q000175:diagnosis; D055815:Young Adult
keywords: bronchiectasis; diagnosis; primary ciliary dyskinesia