The AREST CF program has ethical approval from the Princess Margaret Hospital for Children ethics committee and all parents/legal guardians signed written consent prior to enrollment. Infant lung function testing Infant lung function testing http://www.selleckchem.com/products/ABT-888.html was performed following oral chloral hydrate (60�C100 mg/kg) sedation. Multiple breath washout testing was performed as previously described by our group [11] and others [7], [8], [9], [23], [24] using 5% sulphur-hexafluoride (SF6) as a tracer gas using an ultrasonic flow meter (Ecomedics AG, Duerten, Switzerland). Assessments of lung volume using this technique have been reported to show acceptable agreement when compared to mass spectrometry [25].

Data were included for analysis if there was no evidence of leak or irregular breathing, corrected using an updated temperature model [7], [23], [24] and the effective dead space of the measurement apparatus and face mask [26]. Functional residual capacity (FRC), LCI and the first and second moment ratios (M1/M0 and M2/M0) were derived as reported previously [8], [27]. Chest CT and BAL The chest CT followed by BAL were performed under the same intravenous general anesthesia protocol as previously described [5], [22]. Limited dose three slice chest CT scans (100 kV and 40�C80 mAs) were obtained at a positive pressure of 25 cmH20 and end-expiration (0 cmH20). In March 2007 volumetric helical inspiratory scans (120 kv; 25 mAs; pitch 0.6; total radiation dose=0.74�C0.80 mSv) were introduced as the standard imaging technique, expiratory scans remained unchanged.

In those children with a helical scans, three slices equivalent to the chest CT limited scan were extracted using the scout film to identify the ��slices�� to exactly correspond to the anatomical landmarks used for the limited slice scans. This strategy was used to maximise the number of scans available to compare with MBW outcomes. Immediately following chest CT the bronchoscope was introduced into the lower airway and BAL obtained from three aliquots of 1 mL/kg of sterile saline instilled into the right middle lobe followed by a single instillation into lingual or worst lobe on radiology. The two first aliquots (one right, one lingual) were sent separately for microbiological assessment and the 2nd and 3rd right middle lobe aliquots pooled and processed within 1 hour for subsequent inflammatory analysis.

BAL samples were cultured by standard techniques and infection with a specific organism was considered as ��105 cfu/mL. Samples that cultured mixed oral flora (MOF) and isolated colonies <105 CFU/mL were classified as uninfected. Chest CT scoring Chest CT images were scored by a single experienced pediatric thoracic radiologist (C.M.) in six zones (upper, mid, and lower; right and left) as previously reported by our group [5], [22]. Carfilzomib The presence of bronchiectasis or air-trapping was recorded in a binary fashion for each abnormality for each zone.