Rationale: Lower airway (LAW) infection with Pseudomonas aeruginosa and Staphylococcus aureus is the leading cause of morbidity in cystic fibrosis (CF). The upper airways (UAW) were shown to be a gateway for acquisition of opportunistic bacteria and to act as a reservoir for them. Therefore, tools for UAW assessment within CF routine care require evaluation. Objectives: The aims of the study were non-invasive assessment of UAW and LAW microbial colonisation, and genotyping of P aeruginosa and S aureus strains from both segments. Methods: 182 patients with CF were evaluated (age 0.4-68 years, median 17 years). LAW specimens were preferably sampled as expectorated sputum and UAW specimens by nasal lavage. P aeruginosa and S aureus isolates were typed by informative single nucleotide polymorphisms (SNPs) or by spa typing, respectively. Results: Of the typable S aureus and P aeruginosa isolates from concomitant UAW-and LAW-positive specimens, 31 of 36 patients were carrying identical S aureus spa types and 23 of 24 patients identical P aeruginosa SNP genotypes in both compartments. Detection of S aureus or P aeruginosa in LAW specimens was associated with a 15-or 88-fold higher likelihood also to identify S aureus or P aeruginosa in a UAW specimen from the same patient. Conclusions: The presence of identical genotypes in UAW and LAW suggests that the UAW play a role as a reservoir of S aureus and P aeruginosa in CF. Nasal lavage appears to be suitable for non-invasive UAW sampling, but further longitudinal analyses and comparison with invasive methods are required. While UAW bacterial colonisation is typically not assessed in regular CF care, the data challenge the need to discuss diagnostic and therapeutic standards for this airway compartment. Trial registration number: NCT00266474.

NL-fluid differed substantially between CF-patients and healthy controls with most promising results for IL-8 and MPO, a primarily in CF-NL assessed mediator. Further studies are required to assess effects of sample collection and processing on concentrations of inflammatory markers and to evaluate potentials of NL analysis in research and clinical routine.

Among SNOT-20 scores the PNS subscore is related to rhinoscopically detected polyposis and sinonasal secretion. Therefore, we recommend including short questions regarding PNS into CF-routine care. At the same time our results show that a high inspiratory airflow is not associated with a good sensation of nasal patency. Altogether, rhinomanometry is not required within routine CF-care, but it can be interesting as an outcome parameter within clinical trials. Pediatr Pulmonol. 2017;52:167-174. © 2016 Wiley Periodicals, Inc.

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