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

Respiration ◽  
2018 ◽  
Vol 97 (1) ◽  
pp. 60-69 ◽  
Author(s):  
Andrea Radine ◽  
Claudius Werner ◽  
Johanna Raidt ◽  
Gerard W. Dougherty ◽  
Laura Kerschke ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Observational Study ◽  
Healthy Subjects ◽  
Primary Ciliary Dyskinesia ◽  
Prospective Observational Study ◽  
Nocturnal Cough ◽  
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.

scholarly journals Bacteraemia and fungaemia in cystic fibrosis patients with febrile pulmonary exacerbation: a prospective observational study

2017 ◽  
Vol 17 (1) ◽  
Author(s):  
Joerg Grosse-Onnebrink ◽  
Florian Stehling ◽  
Eva Tschiedel ◽  
Margarete Olivier ◽  
Uwe Mellies ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Observational Study ◽  
Prospective Observational Study ◽  
Pulmonary Exacerbation

Caregiver burden in children with cystic fibrosis and primary ciliary dyskinesia

2019 ◽  
Vol 54 (12) ◽  
pp. 1936-1940 ◽  
Author(s):  
Özge Keniş Coşkun ◽  
Kardelen Gençer Atalay ◽  
Ela Erdem ◽  
Evrim Karadag‐Saygi ◽  
Yasemin Gökdemir ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Caregiver Burden ◽  
Primary Ciliary Dyskinesia ◽  
Ciliary Dyskinesia

scholarly journals Intravenous antibiotic use and exacerbation events in an adult cystic fibrosis centre: A prospective observational study

2019 ◽  
Vol 154 ◽  
pp. 109-115 ◽  
Author(s):  
Zhe Hui Hoo ◽  
Nicole R. Bramley ◽  
Rachael Curley ◽  
Frank P. Edenborough ◽  
Stephen J. Walters ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Observational Study ◽  
Prospective Observational Study ◽  
Antibiotic Use ◽  
Intravenous Antibiotic ◽  
Cystic Fibrosis Centre

Exhaled Molecular Profiles In The Diagnosis Of Cystic Fibrosis And Primary Ciliary Dyskinesia

2012 ◽  
Author(s):  
Tamara Paff ◽  
Marc P. van der Schee ◽  
Johannes M.A. Daniels ◽  
Gerard Pals ◽  
Pieter E. Postmus ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Primary Ciliary Dyskinesia ◽  
Molecular Profiles ◽  
Ciliary Dyskinesia

scholarly journals Computed Tomography Description of the Uncinate Process Angulation in Patients With Cystic Fibrosis and Comparison With Primary Ciliary Dyskinesia, Nasal Polyposis, and Controls

2019 ◽  
Vol 98 (2) ◽  
pp. 89-93 ◽  
Author(s):  
Rémi Hervochon ◽  
Natacha Teissier ◽  
Jean-Robert Blondeau ◽  
Natacha Remus ◽  
Laurence Bassinet ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Cystic Fibrosis ◽  
Primary Ciliary Dyskinesia ◽  
Nasal Polyposis ◽  
Uncinate Process ◽  
Ct Scans ◽  
Lateral Nasal Wall ◽  
Average Value ◽  
Significant Difference ◽  
Ciliary Dyskinesia

Background: There is a medial bulging of the lateral nasal wall in patients with cystic fibrosis (CF). Aims: Uncinate process (UP) angulation measurements in patients and controls to objectify this bulging. Materials and Methods: Thirty CF, 17 primary ciliary dyskinesia (PCD), 13 chronic rhinosinusitis with polyps (CRSwp), and 30 controls were included. Angles were measured bilaterally on computed tomography (CT) scans: A, B, C on coronal sections, D and E on axial sections. Angle A was between the UP and the orbit inner wall, whereas the others were between UP and midline. Results: There was no significant difference between controls, PCD, and CRSwp. However, CF had 3 statistically different angles with controls, 5 with CRSwp, and 4 with PCD. Angle A average value was 126° (±16°) in patients with CF, 138° (±19°) in controls ( P = .007), 145° (±15°) in PCD ( P = .001), and 138° (±14°) in CRSwp ( P = .001). Angle E average value was 35° (±10°) in patients with CF, 20° (±6°) in controls ( P < .001), 21° (±4°) in PCD ( P < .001), and 22° (±6°) in CRSwp ( P < .001). Conclusion: Uncinate process’s anatomy is only modified in CF: Angle between UP and inner wall of orbit is closed, and angles between UP and midline are opened. Significance: These measures quantify the medial bulging of lateral nasal wall and support nasofibroscopic observations.

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