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Primary Ciliary Dyskinesia in Six Patients With Bronchiectasis Publisher Pubmed



Modaresi M1, 2 ; Sadeghi B1, 2 ; Mohammadinejad P1, 2 ; Sayedi SJ1, 2 ; Masiha F1, 2 ; Shirzadi R1, 2 ; Azizi G1, 2, 3 ; Aghamohammadi A1, 2
Authors
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Authors Affiliations
  1. 1. Pediatrics Pulmonary Department, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Pediatrics Pulmonary Department, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  3. 3. Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran

Source: Advances in Respiratory Medicine Published:2015


Abstract

Introduction: Primary ciliary dyskinesia [PCD] is generally considered as a rare autosomal recessive disorder. Previous studies reported various prevalence of PCD among patients with bronchiectasis. Material and 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. © 2016 PTChP.