In PCD patients microbiological positivity is highly prevalent, with Haemophilus influenzae, Staphylococcus aureus, and Pseudomonas aeruginosa the most frequently isolated pathogens; most patients colonized at baseline remained colonized over time, and those colonized by P. aeruginosa tended to exhibit greater bronchiectasis extension and worse lung function.
Most PCD patients had a positive respiratory microbiological status at baseline.
Most colonized patients remained colonized at 12 months, though the isolated microorganism often changed.
Patients with negative sputum at baseline were about half likely to remain negative at 12 months.
Acute infection at baseline had divergent outcomes: equal proportions progressed to colonization or became sputum-negative.
No significant clinical or functional differences were observed among the microbiological status groups (negative, colonized, acute infection).
S. aureus, H. influenzae, and P. aeruginosa were the most commonly isolated pathogens.
Patients colonized with P. aeruginosa had worse lung function and more extensive bronchiectasis.
This study followed 82 children and young adults in Italy with primary ciliary dyskinesia (PCD), a genetic condition in which the airways cannot clear mucus well, leading to repeated lung infections. Researchers checked sputum cultures, lung function, and questionnaires at the start and every three months for a year to see which bacteria were present and how this changed. At the start, only about a quarter of patients had negative sputum cultures; about half were colonized by bacteria and about a quarter had an acute infection. Three-quarters of those colonized stayed colonized after a year, although the specific bacteria often changed. About half of negative patients stayed negative, and patients with an acute infection were split between becoming colonized and clearing the bacteria. The most common bacteria were Staphylococcus aureus, Haemophilus influenzae, and Pseudomonas aeruginosa. Overall, lung function and clinical features did not differ significantly between the groups. However, patients colonized with Pseudomonas aeruginosa tended to have lower lung function and more widespread bronchiectasis (damaged, widened airways). This research suggests that persistent bacterial colonization is common in PCD and that Pseudomonas may be linked to more severe lung disease, supporting regular microbiological monitoring. The study cannot show that the bacteria cause the damage, and its modest size and one-year follow-up limit conclusions.
Ferraro V, Tonazzo V, Pandolfo A, Biondi C, Boni A, Borrelli M, et al.. (2026). Longitudinal Characterization of the Microbiological Status in Primary Ciliary Dyskinesia: An Italian Multicentre Study.. Pediatric pulmonology. https://doi.org/10.1002/ppul.71875