Lung Function in Patients with Primary Ciliary Dyskinesia

Marthin, June Kehlet; Petersen, Nadia; Skovgaard, L. T.; Nielsen, Kim G.

doi:10.1164/rccm.200811-1731oc

Cited by 176 publications

(77 citation statements)

References 14 publications

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“…In all PCD patients, spirometery should be requested and considered as routine follow-up investigations [15]. Lung involvement included obstructive, restrictive and mixed patterns in function, which is compatible with other studies on respiratory outcomes of PCD [7].…”

Section: Discussionsupporting

confidence: 81%

Primary Ciliary Dyskinesia in Six Patients with Bronchiectasis

Modaresi

Sadeghi

Mohammadinejad

et al. 2016

Advances in Respiratory Medicine

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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.

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Section: Discussionsupporting

confidence: 81%

Primary Ciliary Dyskinesia in Six Patients with Bronchiectasis

Modaresi

Sadeghi

Mohammadinejad

et al. 2016

Advances in Respiratory Medicine

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“…The worse lung function and clinical picture of PCD patients who presented to our center in adulthood, underline the need for establishment of diagnosis and initiation of specific treatment as early as possible, well before the beginning of the second decade of life, when undiagnosed PCD patients seem to develop severe lung disease that is difficult to control with conservative treatment. Despite the findings of some recent studies which suggested that early diagnosis does not protect against decline in lung function [25,26], the need of early diagnosis in PCD is critical and based on our data, will help avoid the performance of lobectomies in undiagnosed PCD patients with advanced lung disease.…”

Section: Discussionmentioning

confidence: 65%

“…This finding suggests that lung function was not affected so much by the progression of time but from events that related to loss of function from earlier in life and probably the lobectomies per se. Significant loss of lung function has been demonstrated to appear in PCD patients from early on in life, starting already from the preschool age [25]. Although it cannot be excluded that the lobectomies were performed in those patients who were more severe in the first instance, our findings suggest that lobectomy should be probably avoided in the management of bronchiectasis in PCD patients.…”

Section: Discussionmentioning

confidence: 68%

Clinical features of primary ciliary dyskinesia in Cyprus with emphasis on lobectomized patients

Yiallouros

Kouis

Middleton

et al. 2015

Respiratory Medicine

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“…The cough clearance is preserved in PCD while it is reduced in CF patients, which is probably a result of increased sputum tenacity [39, 40]. These pathophysiological differences likely account for the worse course of lung disease in CF compared to PCD [8, 44-47]. These results are somewhat unexpected based on our clinical observations that patients with PCD cough more than patients with CF, especially in patients with less advanced lung disease.…”

Section: Discussionmentioning

confidence: 94%

Comparison of Nocturnal Cough Analysis in Healthy Subjects and in Patients with Cystic Fibrosis and Primary Ciliary Dyskinesia: A Prospective Observational Study

et al. 2018

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Background: Cough is a key symptom in patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD). Objective: The study objectives were to test whether cough is related to parameters reflecting their disease severity and whether CF and PCD differ in cough frequency. Methods: In this prospective observational study, we used a microphone-based monitoring system (LEOSound® Monitor) to count the coughs in healthy subjects (HS) and in stable patients with CF and PCD (25 subjects per group) on 2 consecutive nights. Results: The median number of coughs/h in the HS, CF, and PCD groups was 0.0, 1.3, and 0.5 on the first night and 0.0, 2.3, and 0.2 on the second night, respectively. Patients with CF and PCD coughed more than HS (p < 0.001 and p = 0.009, respectively) and CF patients coughed more than PCD patients (p = 0.023). A multivariable mixed model analysis revealed forced expiratory volume in 1 s as an independent risk factor for increased cough frequency in patients. The reliability for repeated measurements was higher for cough epochs/h than for coughs/h (intraclass correlation coefficient: 0.75 and 0.49, respectively). Conclusions: Patients with CF cough more than patients with PCD. The cough frequency in CF and PCD is associated with parameters reflecting disease severity. Cough frequency is a possible endpoint in clinical trials and cough epochs/h may be more useful than coughs/h.

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Lung Function in Patients with Primary Ciliary Dyskinesia

Cited by 176 publications

References 14 publications

Primary Ciliary Dyskinesia in Six Patients with Bronchiectasis

Primary Ciliary Dyskinesia in Six Patients with Bronchiectasis

Clinical features of primary ciliary dyskinesia in Cyprus with emphasis on lobectomized patients

Comparison of Nocturnal Cough Analysis in Healthy Subjects and in Patients with Cystic Fibrosis and Primary Ciliary Dyskinesia: A Prospective Observational Study

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