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High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia
Published on: January 19, 2022
Etiological Work-Up for Adults with Bronchiectasis: A Predictive Diagnostic Score for Primary Ciliary Dyskinesia and
Frederic Schlemmer1,2,3, Agnes Hamzaoui4, Sonia Zebachi5
1Service de Pneumologie, Centre Hospitalier Intercommunal, FHU SENEC, F-94000 Créteil, France.
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.
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