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Background: Pulmonary exacerbations in cystic fibrosis (CF) contribute to the burden of disease, with a negative impact on quality of life, costs, and lung function. Our aim was to evaluate whether exacerbations, defi ned by antibiotic use, were triggered by daily fl uctuations in air pollution., Methods: In a case-crossover analysis, we evaluated 215 patients with CF and pollution data from January 1, 1998, to December 31, 2010. Exacerbation was defi ned as the start of IV or oral antibiotic use in a home or hospital setting. We calculated regional background levels of particulate matter with a diameter , 10 m m (PM 10 ), ozone, and nitrogen dioxide (NO 2 ) on the day of the event and on the 2 days prior to the event at each patient’s home address. We matched for day of the week and controlled for temperature on the day of the event and the 2 preceding days. In the month where antibiotic treatment was started, all days with the same temperature ( 2°C) as the event day served as control days, excluding 3 days before and after the start of treatment., Results: A total of 215 patients (male sex, 49%, mean age, 21 13 years) had 2,204 antibiotic treatments (1,107 IV and 1,097 oral). Over a period of 12 years, an increase in risk of antibiotic use was associated with increasing concentrations of PM 10 , NO 2 , and ozone on the event day and for NO 2 on the day before. A tendency toward signifi cance was seen the day before antibiotic use for PM 10 and ozone. Overall, a rise in OR was seen from 2 days before until the day of the start of antibiotics., Conclusions: In patients with CF and exacerbations, ambient concentrations of ozone, PM 10 , and NO 2 play a role in triggering an exacerbation.

Study Objectives: The aim of the present study was to evaluate the forced oscillation technique (FOT) in cystic fibrosis (CF) children and to participate in the discussion about the usefulness of beta2-antagonists in CF., Design: Pulmonary function was measured with spirometry, body plethysmography, and FOT before and after inhalation of 200 microg of albuterol (salbutamol). The following were collected: vital capacity (VC), FEV1, FEV1/VC, airway resistance (Raw), thoracic gas volume, respiratory system resistance (Rrs) and respiratory system reactance (Xrs) at 6 Hz (Rrs6 and Xrs6), and resonance frequency., Setting: The study was set up at a university hospital with a CF population of 125 children and adolescents., Patients: Data were collected on 20 patients in stable condition able to perform the three lung function tests., Measurements and Results: Mean baseline values (+/-SD) were 0.36+/-0.15 kPa/L/s for Raw, 0.5+/-0.15 kPa/L/s for Rrs6, and 61+/-22% predicted for FEV1. The relationship between FEV1 and Raw or Rrs6 was poor. Xrs6 and FEV1/VC correlated weakly (r=0.56; p < 0.05). After bronchodilator administration, the mean changes +/-SD in percent of baseline were +3 +/- 11% for FEV1, -16 +/- 22% for Raw, and -16 +/- 9% for Rrs6. In six patients, a paradoxical decrease in FEV1 was measured but an increase in Rrs6 was never found; in two patients, an increase of Raw of < 10% was found. In 13 patients, the decrease of Rrs6 was > 12%., Conclusions: The results suggest that FOT measurements cannot replace baseline spirometric measurements in CF, but that the evaluation of the effect of beta2-agonists on the airway diameter in CF should include an FOT measurement.

The long-term pulmonary consequences of right middle lobe syndrome (RMLS) in childhood are not known. Therefore, outcome was evaluated in 17 children with RMLS diagnosed in early childhood (mean age, 3.3 years; SD, 1.1 year). Mean age at follow-up was 10.1 years (SD, 2.6 years). RMLS was defined as atelectasis of the right middle lobe (RML) of at least 1 month's duration and visible on the lateral view of the chest radiograph as a wedge-shaped density extending from the hilum anteriorly and downward. Seventeen children without personal history of allergy or respiratory tract disease were studied as control group. Five of 17 study group children had ongoing respiratory problems: symptoms of asthma were present in 4 patients, and cylindrical bronchiectasis was present in one patient. Chest radiograph at follow-up was abnormal in six patients. Pulmonary function tests, including mean and SEM for vital capacity (VC) (82% of predicted +/- 7 vs 94% predicted +/- 3), FEV1 (77% of predicted +/- 12 vs 96% of predicted +/- 4) and their ratio (75 +/- 5 vs 85 +/- 3) were significantly lower in patients with ongoing respiratory symptoms than in the control children. The provocative dose causing a 20% decrease in FEV1 (PD20) of methacholine was significantly lower in patients with ongoing symptoms at follow-up than in control children and in patients without symptoms at follow-up (2.8[2.2 to 3.1] vs 4.5[2.2 to 8.8] and 9.2[2.3 to 24] mg/mL; median and P25-75, p < 0.05). Age at initial diagnosis tended to be younger in patients with ongoing symptoms at follow-up (2.3 +/- 0.7 years vs 3.8 +/- 0.4 years; p < 0.08).