The prevalence of primary ciliary dyskinesia (PCD) in adult patients with bronchiectasis in Dnipro (Ukraine)

2021 ◽  
Author(s):  
Kseniia Suska ◽  
Kateryna Gashynova ◽  
Valeriia Dmytrychenko
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Adult Patients ◽  
Ciliary Dyskinesia

scholarly journals Etiological Work-Up for Adults with Bronchiectasis: A Predictive Diagnostic Score for Primary Ciliary Dyskinesia and Cystic Fibrosis

2021 ◽  
Vol 10 (16) ◽  
pp. 3478
Author(s):  
Frederic Schlemmer ◽  
Agnes Hamzaoui ◽  
Sonia Zebachi ◽  
Aurelie Le Thuaut ◽  
Gilles Mangiapan ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Primary Ciliary Dyskinesia ◽  
Clinical Score ◽  
Adult Patients ◽  
Operating Characteristics ◽  
Diagnostic Score ◽  
Receiver Operating Characteristics Curve ◽  
Diagnostic Work ◽  
Work Up ◽  
Ciliary Dyskinesia

Background: etiological investigations are not done for all adult patients with bronchiectasis because of the availability and interpretation of tests. The aim of the study was to elaborate a score to identify patients at high risk of having cystic fibrosis or primary ciliary dyskinesia (CF/PCD), which require appropriate management. Methods: diagnostic work-ups were carried out on a French monocenter cohort, and results were subjected to logistic-regression analyses to identify the independent factors associated with CF/PCD diagnosis and, thereby, elaborate a score to validate in a second cohort. Results: among 188 patients, 158 had no obvious diagnosis and were enrolled in the algorithm-construction group. In multivariate analyses, age at symptom onset (8.69 (2.10–35.99); p = 0.003), chronic ENT symptoms or diagnosed sinusitis (10.53 (1.26–87.57); p = 0.03), digestive symptoms or situs inversus (5.10 (1.23–21.14); p = 0.025), and Pseudomonas. aeruginosa and/or Staphylococcus aureus isolated from sputum (11.13 (1.34–92.21); p = 0.02) are associated with CF or PCD. Receiver operating characteristics curve analysis, using a validation group of 167 patients with bronchiectasis, confirmed the score’s performance with AUC 0.92 (95% CI: 0.84–0.98). Conclusions: a clinical score may help identify adult patients with bronchiectasis at higher risk of having CF or PCD.

Primary ciliary dyskinesia diagnosis in adult patients with bronchiectasis in Sao Paulo, Brazil.

2018 ◽  
Author(s):  
Mary Anne Kowal Olm ◽  
Rodrigo Abensur Athanazio ◽  
Samia Rached ◽  
Niki Tomas Loges ◽  
Heymut Omran ◽  
...  
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Sao Paulo ◽  
Adult Patients ◽  
São Paulo ◽  
Ciliary Dyskinesia

Vestibular function in adult patients with primary ciliary dyskinesia

2021 ◽  
Author(s):  
Niels Otto Stahl Winthereik ◽  
Mikkel Christian Alanin ◽  
Kim Gjerum Nielsen ◽  
Christian Von Buchwald ◽  
Sune Land Bloch
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Vestibular Function ◽  
Adult Patients ◽  
Ciliary Dyskinesia

Hypofertility in adult patients with primary ciliary dyskinesia

2017 ◽  
Author(s):  
Bernard Maitre ◽  
Gert Jan Vanaken ◽  
Laurence Bassinet ◽  
Mieke Boon ◽  
Rahma Mani ◽  
...  
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Adult Patients ◽  
Ciliary Dyskinesia

scholarly journals Follow-Up and Management of Chronic Rhinosinusitis in Adults with Primary Ciliary Dyskinesia: Review and Experience of Our Reference Centers

2019 ◽  
Vol 8 (9) ◽  
pp. 1495 ◽  
Author(s):  
Emilie Bequignon ◽  
Laurence Dupuy ◽  
Virginie Escabasse ◽  
Francoise Zerah-Lancner ◽  
Laurence Bassinet ◽  
...  
Keyword(s):  
Chronic Rhinosinusitis ◽  
Primary Ciliary Dyskinesia ◽  
Adult Patients ◽  
Middle Meatus ◽  
Endoscopic View ◽  
Therapy Strategy ◽  
Significant Difference ◽  
Diagnostic Work ◽  
Surgical Treatments ◽  
Ciliary Dyskinesia

Chronic rhinosinusitis is the foremost manifestation in adult patients with primary ciliary dyskinesia (PCD). We present a retrospective series of 41 adult patients with a confirmed diagnosis of PCD followed in our reference centers. As part of the diagnostic work up in our centers, sinus computed tomography scans (CTs) are systematically performed. All patients also undergo a sampling of purulent secretions sampled from the middle meatus under endoscopic view for bacteriological analysis. In our series, CT opacities were consistent in all the patients, as well as mainly partial and located in ethmoid cells (100% of patients) and in maxillary sinuses (85.4% of patients), and stayed stable over time. In the 31 patients who had purulent secretions, bacteriological culture showed at least one bacterium in 83.9% (n = 26). There was no significant difference in positive cultures for Pseudomonas aeruginosa in patients >40 years old versus those <40 (p = 0.17; Fisher). Surgical management was performed in only 19% of patients in order to improve sinonasal mechanical drainage. Our data support the hypothesis that the sinuses can be considered as a bacterial reservoir. From this retrospective study, we have introduced several changes into our routine clinical practice in our reference centers. Based on our analyses, medical and surgical treatments benefit from incorporating bacteriological information and sinonasal symptoms much more than CT scan evaluation alone. All patients now undergo systematically an annual simultaneous bacteriological sampling of the middle meatus and sputum to follow the relationship between ENT and lung disease and to help to antibiotic therapy strategy.

scholarly journals Clinical characteristics, functional respiratory decline and follow-up in adult patients with primary ciliary dyskinesia

Thorax ◽  
2016 ◽  
Vol 72 (2) ◽  
pp. 154-160 ◽  
Author(s):  
Justine Frija-Masson ◽  
Laurence Bassinet ◽  
Isabelle Honoré ◽  
Nadine Dufeu ◽  
Bruno Housset ◽  
...  
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Clinical Characteristics ◽  
Adult Patients ◽  
Ciliary Dyskinesia

scholarly journals Why, when and how to investigate primary ciliary dyskinesia in adult patients with bronchiectasis

2019 ◽  
Author(s):  
Martina Contarini ◽  
Amelia Shoemark ◽  
Jessica Rademacher ◽  
Simon Finch ◽  
Andrea Gramegna ◽  
...  
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Legal Aspects ◽  
Adult Patients ◽  
Diagnostic Approach ◽  
Screening Tests ◽  
Team Approach ◽  
Number Of Patients ◽  
Missed Diagnosis ◽  
High Level ◽  
Ciliary Dyskinesia

Bronchiectasis represents the final pathway of several infectious, genetic, immunologic or allergic disorders. Accurate and prompt identification of the underlying cause is a key recommendation of several international guidelines, in order to tailor treatment appropriately. Primary ciliary dyskinesia (PCD) is a genetic cause of bronchiectasis in which failure of motile cilia leads to poor mucociliary clearance. Due to poor ciliary function in other organs, individuals can suffer from chronic rhinosinusitis, otitis media and infertility. This paper explores the current literature describing why, when and how to investigate PCD in adult patients with bronchiectasis. We describe the main PCD diagnostic tests and compare the two international PCD diagnostic guidelines. The expensive multi-test diagnostic approach requiring a high level of expertise and specialist equipment, make the multifaceted PCD diagnostic pathway complex. Therefore, the risk of late or missed diagnosis is high and has clinical and research implications. Defining the number of patients with bronchiectasis due to PCD is complex. To date, few studies outlining the aetiology of adult patients with bronchiectasis conduct screening tests for PCD, but they do differ in their diagnostic approach. Comparison of these studies reveals an estimated PCD prevalence of 1–13% in adults with bronchiectasis and describe patients as younger than their counterparts with moderate impairment of lung function and higher rates of chronic infection with Pseudomonas aeruginosa. Diagnosing PCD has clinical, socioeconomic and psychological implications, which affect patients’ life, including the possibility to have a specific and multidisciplinary team approach in a PCD referral centre, as well as a genetic and fertility counselling and special legal aspects in some countries. To date no specific treatments for PCD have been approved, standardized diagnostic protocols for PCD and recent diagnostic guidelines will be helpful to accurately define a population on which planning RCT studies to evaluate efficacy, safety and accuracy of PCD specific treatments.

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