Objectives To assess the effects of Pseudomonas aeruginosa (Pa) and Staphylococcus aureus (Sa) infection on lower airway inflammation and clinical status in young children with cystic fibrosis (CF). Study design We studied 111 children < 6 years of age who had two Pa positive oropharyngeal cultures within 12 months. We examined bronchoalveolar lavage fluid (BALF) inflammatory markers (cell count, differential, IL-8, IL-6, neutrophil elastase), CF-related bacterial pathogens, exotoxin A serology, and clinical indicators of disease severity. Results Young children with CF with both upper and lower airway Pa infection had higher neutrophil counts, IL-8 and free neutrophil elastase levels, increased likelihood of positive exotoxin A titers, and lower Shwachman scores compared with those with positive upper airway cultures only. Sa was associated with increased lower airway inflammation and the presence of both Pa and Sa had an additive effect on concentrations of lower airway inflammatory markers. BALF markers of inflammation increased with numbers of different bacterial pathogens detected. Conclusions Young children with CF with upper and lower airway Pa infection have heightened endobronchial inflammation and poorer clinical status compared with children with only upper airway Pa infection. The independent and additive effects of Sa on inflammation support the importance of polymicrobial infection in early CF lung disease.

Study findings are suggestive of age-related differences in adherence behaviors across both IC and medical regimens and support the use of developmentally sensitive approaches to assessment and interventions addressing adherence.

These findings provide further support for the HBM in evaluating pediatric adherence, with evidence that barriers and cues to action may be targets for early intervention. Future research using this model to identify a comprehensive way to assess, understand, and elicit change in the adherence to medical regimens for youth with chronic illness would be beneficial.

The goal of this research was to begin the process of evaluating acceptability of infection control (IC) recommendations to CF patients and their families, determine whether compliance with IC guidelines differs from compliance with traditional CF medical treatment with respect to the variables predictive of compliance, and assess which patients are most likely to comply with IC recommendations. Participants were recruited during routine outpatient visits at a regional CF center located in a pediatric hospital. The sample included 44 child and adolescent patients, aged 9-18 years and their guardian, and 27 adult patients. All patients completed questionnaires and interviews. Results of this preliminary study suggest that many individuals with CF are unaware of or unconcerned with the risks involved in infection transmission via social contact with other CF patients. Further, most participants reported that they could benefit from friendships with other CF patients. Health belief variables were found to be predictive of compliance with both IC guidelines and traditional medical treatments in the adult and parent sample, but not in the child sample. Possible explanations for study findings are discussed and recommendations for future research on IC compliance are highlighted.

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