Longitudinal Characterization of the Microbiological Status in Primary Ciliary Dyskinesia: An Italian Multicentre Study
Valentina Agnese Ferraro, Valentina Tonazzo, Alessandra Pandolfo, Claudia Biondi, Alessandra Boni, Melissa Borrelli, Claudia Calogero, Alberta Circiello, Adele Corcione, Renato Cutrera, Valentina Fainardi, Giuseppe Fabio Parisi, Maria Francesca Patria, Giuseppe Scopelliti, Nicola Ullmann, Silvia CarraroABSTRACT
Background and Aim
Primary Ciliary Dyskinesia (PCD) is a genetic disorder characterized by respiratory infections leading to chronic airway colonization and progressive lung damage. The aim of this study is to evaluate prevalence and longitudinal changes of respiratory microbiological profile in children and young adults with PCD, and their association with clinical, functional, and radiological features.
Materials and Methods
This prospective, multicenter longitudinal study included PCD patients from seven Italian Pediatric Pulmonology centers within the SIMRI (Italian Pediatric Respiratory Society) “PCD and Non‐CF Bronchiectasis” study group. Patients were evaluated at baseline (T0) with questionnaires, spirometry, and sputum cultures, and followed for 12 months, with quarterly assessments.
Results
82 patients were enrolled (mean age 16.1 ± 5.9 y). At T0, 21 had negative sputum, 41 were colonized, 20 had acute infection. After 12 months, 75% of colonized patients remained colonized, although the microorganism identified during follow‐up was not necessarily the same as that detected at baseline, and 52% of negative patients remained negative. Among acute infection, 40% progressed to colonization and 40% became sputum‐negative. No significant clinical or functional differences were observed among microbiological groups. The most common pathogens were S. aureus (acute 18%, colonization 33%), H. influenzae (acute 14%, colonization 33%), and P. aeruginosa (acute 9%, colonization 23%). Patients colonized with P. aeruginosa showed lower spirometric values and more extensive bronchiectasis.
Conclusions
In PCD patients microbiological positivity is highly prevalent, being Haemophilus influenzae , Staphylococcus aureus , and Pseudomonas aeruginosa the most frequently isolated pathogens. Most patients colonized at baseline remained colonized over time and patients colonized by Pseudomonas aeruginosa tended to exhibit greater bronchiectasis extension and worse lung function.