open access

Vol 84, No 2 (2016)
CASE REPORTS
Submitted: 2016-03-23
Get Citation

Primary ciliary dyskinesia in six patients with bronchiectasis

Mohammadreza Modaresi, Bamdad Sadeghi, Payam Mohammadinejad, Sayed Javad Sayedi, Farzad Masiha, Rohola Shirzadi, Gholamreza Azizi, Asghar Aghamohammadi
DOI: 10.5603/PiAP.2016.0009
·
Pneumonol Alergol Pol 2016;84(2):109-115.

open access

Vol 84, No 2 (2016)
CASE REPORTS
Submitted: 2016-03-23

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.

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.

Get Citation

Keywords

bronchiectasis, diagnosis, primary ciliary dyskinesia

About this article
Title

Primary ciliary dyskinesia in six patients with bronchiectasis

Journal

Advances in Respiratory Medicine

Issue

Vol 84, No 2 (2016)

Pages

109-115

DOI

10.5603/PiAP.2016.0009

Bibliographic record

Pneumonol Alergol Pol 2016;84(2):109-115.

Keywords

bronchiectasis
diagnosis
primary ciliary dyskinesia

Authors

Mohammadreza Modaresi
Bamdad Sadeghi
Payam Mohammadinejad
Sayed Javad Sayedi
Farzad Masiha
Rohola Shirzadi
Gholamreza Azizi
Asghar Aghamohammadi

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

Czasopismo Pneumonologia i Alergologia Polska dostęne jest również w Ikamed - księgarnia medyczna

Wydawcą serwisu jest "Via Medica sp. z o.o." sp.k., ul. Świętokrzyska 73, 80–180 Gdańsk

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail: viamedica@viamedica.pl