Clinical impact of Pseudomonas aeruginosa colonization in patients with Primary Ciliary Dyskinesia

Cohen‐Cymberknoh, Malena; Weigert, Nir; Gileles‐Hillel, Alex; Breuer, Oded; Simanovsky, Natalia; Boon, Mieke; Boeck, K. De; Barbato, Angelo; Snijders, Deborah; Collura, Mirella; Pradal, Ugo; Blau, Hannah; Mussaffi, Huda; Price, Mareike; Bentur, Lea; Gur, Michal; Aviram, Micha; Picard, Elie; Shteinberg, Michal; Livnat, Galit; Rivlin, Joseph; Hiller, Nurith; Shoseyov, David; Amirav, Israel; Kerem, Eitan

doi:10.1016/j.rmed.2017.08.028

Cited by 39 publications

(50 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…About the results of microbiology of sputum, they resulted in agreement with the results of other studies that reported Haemophilus influentiae and Pseudomonas aeruginosa as the most common pathogens in PCD [12,18].…”

Section: Discussionsupporting

confidence: 88%

“…Considering both pediatric and adult PCD patients, Cohen Cymberknoh [18] also reported an overall prevalence of PA colonization of about 27%; those colonized with PA were older and diagnosed at a later age. Similarly, we observed that chronic PA lung colonization in PCD was associated with older age.…”

Section: Discussionmentioning

confidence: 96%

“…Chronic bronchial infection was defined as the isolation of the same pathogen in sputum culture on two or more occasions, at least 3 months apart in a 1-year period [36]. Similarly, regarding PA colonization, we classified patients as non-colonized if they had never been cultured or cultured only once with this pathogen, and as colonized patients if they showed at least two positive sputum cultures for PA in 1 year (3 months apart) [18,36].…”

Section: Microbiological Datamentioning

confidence: 99%

“…PA is an important pathogen in PCD, with an overall reported prevalence ranging between 27 and 35% [17]. In PCD the presence of PA colonization appears to increase with age [12,17,18].…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Exacerbations and Pseudomonas aeruginosa colonization are associated with altered lung structure and function in primary ciliary dyskinesia

et al. 2020

View full text Add to dashboard Cite

Background: Recurrent bacterial infections of the respiratory tract are one of the major clinical features of the primary ciliary dyskinesia (PCD), a rare genetic disease due to malfunctioning of motile cilia. Chronic infections and persistent inflammation of the respiratory system result in progressive lung disease. Aim of the study was to highlight the main factors associated with clinical, functional and anatomical deterioration in PCD patients. Methods: We retrospectively analyzed data from 58 patients with PCD, 37 adults and 21 children. The demographic and clinical data, forced expiratory volume at 1 s (FEV 1) and forced vital capacity (FVC), sputum microbiology and imaging results (chest CT scores-modified Bhalla) were recorded. Patients were stratified according to the number of exacerbations (< 2/year vs ≥ 2/year) and chronic Pseudomonas aeruginosa (PA) colonization. The possible correlations between lung function and chest CT scores were assessed; we also evaluated the correlation between these parameters and the severity scores for bronchiectasis (BSI, FACED and e-FACED). Results: Chest CT scores showed a significant correlation with FEV 1 (p = 0.0002), age (p < 0.0001), BMI (p = 0.0002) and number of lung lobes involved (p < 0.0001). PA colonization had an overall prevalence of 32.6%: no significant difference in FEV 1 between PA colonized and non-colonized patients was found (p = 0.70), while chest CT score was significantly worse in chronic PA colonized patients (p = 0.009). Patients with a high number of exacerbation (≥ 2/ year) were older (p = 0.01), had lower FEV 1 (p = 0.03), greater number of lobes involved (p < 0.001) and worse CT score than patients with low number of exacerbations (p = 0.001); they also had higher prevalence of PA chronic bronchial infection (33.3% versus 13.6%, p = 0.10). Multivariable linear regression analyses adjusted for gender, age and BMI showed positive associations between PA colonization and number of exacerbations with severity of disease (number of lobes involved, CT score, BSI, FACED, and e-FACED).

show abstract

Section: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 96%

Section: Microbiological Datamentioning

confidence: 99%

“…PA is an important pathogen in PCD, with an overall reported prevalence ranging between 27 and 35% [17]. In PCD the presence of PA colonization appears to increase with age [12,17,18].…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Exacerbations and Pseudomonas aeruginosa colonization are associated with altered lung structure and function in primary ciliary dyskinesia

et al. 2020

View full text Add to dashboard Cite

show abstract

“…We carried out univariate analyses to evaluate the association between various clinical parameters and the total number of coughs/h and cough epochs/h using negative binomial mixed models [19]. Little evidence exists about the associations of clinical parameters with advanced lung disease in PCD patients [20, 21]. In contrast, several parameters are associated with worse course of lung disease or increased mortality in CF patients [22-26].…”

Section: Methodsmentioning

confidence: 99%

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

View full text Add to dashboard Cite

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.

show abstract

Chronic airway disease in primary ciliary dyskinesia—spiced with geno–phenotype associations

Nielsen

Holgersen

Crowley

et al. 2022

American J of Med Genetics Pt C

View full text Add to dashboard Cite

Primary ciliary dyskinesia (PCD) can be defined as a multiorgan ciliopathy with a dominant element of chronic airway disease affecting the nose, sinuses, middle ear, and in particular, the lower airways. Although most patients with PCD are diagnosed during preschool years, it is obvious that the chronic lung disease starts its course already from birth. The many faces of the clinical picture change, as does lung function, structural lung damage, the burden of infection, and of treatment throughout life. A markedly severe neutrophil inflammation in the respiratory tract seems pervasive and is only to a minimal extent ameliorated by a treatment strategy, which is predominantly aimed at bacterial infections. An ever-increasing understanding of the different aspects, their interrelationships, and possible different age courses conditioned by the underlying genotype is the focus of much attention. The future is likely to offer personalized medicine in the form of mRNA therapy, but to that end, it is of utmost importance that all patients with PCD be carefully characterized and given a genetic diagnosis. In this narrative review, we have concentrated on lower airways and summarized the current understanding of the chronic airway disease in this motile ciliopathy. In addition, we highlight the challenges, gaps, and opportunities in PCD lung disease research.

show abstract

Clinical impact of Pseudomonas aeruginosa colonization in patients with Primary Ciliary Dyskinesia

Abstract: Lung PA colonization in PCD is associated with more severe disease as shown by the FEV and CT score. However, the magnitude of decline in pulmonary function was similar in colonized and non-colonized PCD patients.

Cited by 39 publications

References 27 publications

Exacerbations and Pseudomonas aeruginosa colonization are associated with altered lung structure and function in primary ciliary dyskinesia

Exacerbations and Pseudomonas aeruginosa colonization are associated with altered lung structure and function in primary ciliary dyskinesia

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

Chronic airway disease in primary ciliary dyskinesia—spiced with geno–phenotype associations

Contact Info

Product

Resources

About