To assess the occurrence of sleepdisordered breathing, hypoxemia, and sleep architecture in a cohort of infants and children with cystic fibrosis (CF) and normal or mildly impaired lung function in stable clinical condition. Design: Case-control study. Setting: Cystic Fibrosis Unit of a university hospital and pediatric sleep laboratory. Participants: A total of 40 children (aged 6 months to 11 years) with CF in stable condition and 18 healthy agematched control subjects. Intervention: Nocturnal sleep and cardiorespiratory monitoring was performed using a full polysomnographic recording in a sleep laboratory. Main Outcomes Measures: Sleep architecture and respiratory variables. Results: Although awake oxyhemoglobin saturation (SaO 2) values were similar in the 2 groups (98%), the CF group had significantly lower values of nocturnal mean SaO 2. The apnea-hypopnea index was significantly higher in the CF group compared with the controls (mean [SE], 7.3 [1.3] vs 0.5 [0.4], respectively, P Ͻ .001), particularly in preschool-aged children and in children with upper airway abnormalities. In addition, 28 (70%) of the 40 children with CF had mild to moderate obstructive sleep apnea (defined as an apnea-hypopnea index Ͼ2). Children with CF compared with controls also had reduced sleep efficiency (CF group vs controls mean [SE], 80% [41%] vs 88% [13.1%], P Ͻ.001), rapid eye movement sleep duration (11% [0.9%] vs 13% [1%], PϽ.05), and increased number of arousals per hour (11.0 [10] vs 8.2 [0.7], P Ͻ.001). Conclusions: This study showed an early occurrence of obstructive sleep apnea in children with CF in stable condition, associated with a mild level of sleep disruption. Early routine nocturnal respiratory monitoring is advised in children with CF.

In patients with cystic fibrosis (CF), there is an increasing incidence of some uncommon respiratory pathogens, such as Burkholderia cepacia complex (Bcc), Stenotrophomonas maltophilia, and Achromobacter xylosoxidans. In order to evaluate the prevalence and the clinical impact of these pathogens, we retrospectively studied a total of 109 patients followed in our center from 1996 to 2006 and reviewed the results of 1,550 sputum samples. The isolation of Pseudomonas aeruginosa slightly decreased over the observed decade, whereas Staphylococcus aureus exhibited an irregular trend. Infection with Bcc reached a peak in 1998 and successively decreased to a stable 4%. S. maltophilia and A. xylosoxidans were the real emerging pathogens, since first isolation occurred in 2004; however, the percentage of infected patients remained low (7% and 3.2%, respectively) through the years. In conclusion, in our center for CF, the reduced prevalence of P. aeruginosa over the last decade has been associated with a concurrent reduction of infections by Bcc and, as compared to other centers in Italy, Europe, and the US, with a low incidence of emerging pathogens such as S. maltophilia and A. xylosoxidans.

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Methicillin-resistant Staphylococcus aureus (MRSA) infections are increasingly being reported among cystic fibrosis (CF) populations worldwide. In this paper, we sought to examine at the epidemiology, the molecular characterisation and the antibiotic resistance of MRSA isolates in our cohort of CF patients. All MRSA strains were collected prospectively at the University Hospital of Catania, Italy, during a two-year study between mid 2005 to mid 2007 and underwent molecular, pathotype and susceptibility characterisations. Our study demonstrates persisting infections with both hospital-associated (HA-) and community-associated (CA-)MRSA, including Panton-Valentine leukocidin (PVL)-positive strains, in our CF population with an overall prevalence of 7.8%. We demonstrated that, in these patients, persistence was sustained by either identical clones that underwent subtle changes in their toxin content or by different clones over time. The isolation of MRSA in our CF population aged 7-24 years was associated with an increased severity of the disease even if, due to the small sample of patients included and the paucity of data on the clinical outcome, these results cannot be conclusive. Furthermore, three strains were heteroresistant vancomycin-intermediate S. aureus (hVISA), questioning the use of glycopeptides in the treatment of MRSA infections in these patients.

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