Importance to question sinonasal symptoms and to perform rhinoscopy and rhinomanometry in cystic fibrosis patients.

OBJECTIVES: Cystic fibrosis (CF) patients almost regularly reveal sinonasal pathology. The purpose of this study was to assess association between objective and subjective measurements of sinonasal involvement comparing nasal airflow obtained by active anterior rhinomanometry (AAR), nasal endoscopic findings, and symptoms assessed with the Sino-Nasal Outcome Test-20 (SNOT-20). METHODS: Nasal cavities were explored by anterior rigid rhinoscopy and findings were compared to inspiratory nasal airflow measured by AAR to quantify nasal patency and subjective health-related quality of life in sinonasal disease obtained with the SNOT-20 questionnaire. Relations to upper and lower airway colonization with Pseudomonas aeruginosa, medical treatment, and sinonasal surgery were analysed. RESULTS: A total of 124 CF patients were enrolled (mean age 19.9 ± 10.4 years, range 4-65 years). A significant association of detection of nasal polyposis (NP) in rhinoscopy was found with increased primary nasal symptoms (PNS) which include "nasal obstruction," "sneezing," "runny nose," "thick nasal discharge," and "reduced sense of smell." At the same time patients with pathologically decreased airflow neither showed elevated SNOT-20 scores nor abnormal rhinoscopic findings. Altogether, rhinomanometric and rhinoscopic findings are not significantly related. CONCLUSIONS: 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.

Concordant genotype of upper and lower airways P aeruginosa and S aureus isolates in cystic fibrosis.

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.