No abstract

Background: Non-cystic fibrosis (non-CF) bronchiectasis often starts in childhood with a significant impact on adult morbidity. Little is known about disease progression through childhood and the effect on growth and spirometry. This study reviews longitudinal lung function and growth in children with non-CF bronchiectasis. Methods: The case notes of patients with non-CF bronchiectasis were reviewed retrospectively. Patients were included if at least three calendar years of lung function data were available. Anthropometric measurements and annual spirometry were analysed over both two and four consecutive years. Changes over time were assessed using Generalised Estimating Equations. Results: Fifty-nine patients (31 boys) were identified. At baseline the median age was 8.2 years (range 4.8-15.8), the mean (SD) for height, weight and body mass index (BMI) for age z-scores were 20.68 (1.31), 20.19 (1.34) and 0.19 (1.38), respectively. At baseline, the mean (SD) z-score for forced expiratory volume in 1 s (FEV 1 ) was 22.61 (1.82). Over 2 years (n = 59), mean FEV 1 and forced vital capacity (FVC) improved by 0.17 (95% CI 0.01 to 0.34, p = 0.039) and 0.21 (95% CI 0.04 to 0.39, p = 0.016) z-scores per annum, respectively. Over 4 years there was improvement in height-for-age z-scores (slope 0.05, 95% CI 0.01 to 0.095, p = 0.01) but no improvement in other anthropometric variables. There was no change in spirometry (FEV 1 slope 0.00, 95% CI 20.09 to 0.09, p = 0.999 and FVC slope 0.09, 95% CI 20.09 to 0.1, p = 0.859). Conclusions: Children with non-CF bronchiectasis show adequate growth over time. Lung function stabilises but does not normalise with treatment, underscoring the need for early detection and institution of appropriate therapy.

Nitrotyrosine is high in EBC of asthmatic children and could be considered as a noninvasive marker of nitrosative events in the airways.

The functional outcome in preschool severe wheezers with eosinophilic airway inflammation and increased reticular basement membrane (RBM) thickness is unknown. We investigated the relationship between airway pathology at age 2 yrs and lung function at age 4-6 yrs in previous severe wheezers.Severe wheezers previously investigated by endobronchial biopsy and healthy children aged 4-6 yrs were recruited. Lung clearance index (LCI), conducting zone ventilation inhomogeneity (Scond), acinar ventilation inhomogeneity by multiple-breath washout, plethysmographic-specific airway resistance and exhaled nitric oxide fraction (FeNO) were measured. Lung function was compared between wheezers and healthy controls, and in wheezers correlated with past RBM thickness and mucosal eosinophilia (EG2+ cells).72 healthy controls and 28 previous severe wheezers were tested. Wheezers had significantly higher median LCI (6.8 versus 6.6; p50.001) and Scond (0.046 versus 0.016; p,0.0005) than healthy controls. Past RBM thickness (r50.474, p50.047) and EG2+ cells (r50.552, p50.041) showed significant correlations with current FeNO, but no correlations were seen between past RBM thickness and current lung function.RBM thickness and EG2+ cells at age 2 yrs show a significant positive association with FeNO at age 5 yrs. Although lung function was abnormal at age 5 yrs in severe wheezers, this did not correlate with past RBM thickness.

Knowledge of short-and longer-term repeatability of lung function in health and disease is essential to determine bronchodilator reversibility thresholds and to recognise if changes in lung function represent disease progression, therapeutic intervention or normal variability.Multiple-breath washout indices (lung clearance index, conductive ventilation inhomogeneity (Scond)) and specific airway resistance (sRaw) were measured in healthy children and stable wheezers. Measurements were performed at baseline and after 20 min without intervention to assess repeatability and determine bronchodilator reversibility thresholds. Bronchodilator reversibility was assessed by repeating baseline measurements 20 min after inhaled salbutamol. 28 healthy controls, mean¡SD age 6.1¡0.7 years and 62 wheezers 5.4¡0.6 years were tested. Baseline variability in multiple-breath washout indices and sRaw was not significantly different between wheezers and healthy controls. Significant bronchodilator reversibility was only observed in wheezers for Scond (16%), but in both wheezers (37%) and healthy controls (20%) for sRaw. Some wheezers and healthy controls demonstrated increases in multiple-breath washout indices post-bronchodilator.Lung clearance index and sRaw demonstrate low baseline variability in healthy and diseased subjects. Neither multiple-breath washout indices nor sRaw are ideal for assessing bronchodilator reversibility in young children with stable wheeze. These findings will help to interpret the effect of therapeutic interventions in children with respiratory diseases. @ERSpublications Multiple-breath washout indices and sRaw do not reflect bronchodilator reversibility in young children with stable wheeze