Non-Cystic Fibrosis Bronchiectasis in Childhood: Longitudinal Growth and Lung Function

Cristina M Bastardo ¹, Samatha Sonnappa ^2*, Sanja Stanojevic ¹, Aldo Navarro ³, Pedro Mondejar Lopez ¹, Adam Jaffe ² and Andrew Bush ⁴

¹ Institute of Child Health, United Kingdom
² Great Ormond Street Hospital for Children NHS Trust and Institute of Child Health, United Kingdom
³ Royal Brompton Hospital NHS Trust, United Kingdom
⁴ Royal Brompton Hospital NHS Trust and Imperial College London, United Kingdom

^* To whom correspondence should be addressed. E-mail: s.sonnappa{at}ich.ucl.ac.uk.

Accepted 7 November 2008

Abstract

Background: Non-Cystic Fibrosis (non-CF) bronchiectasis often starts in childhood with 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 2 and 4 consecutive years. Changes over time were assessed using Generalized Estimating Equations.

Results: Fifty-nine patients (31 males) were identified. At baseline, the median age was 8.2y (range 4.8-15.8y), the mean ±(SD) for height, weight and body mass index (BMI) for-ages z-scores were -0.68±1.31, -0.19±1.34 and 0.19±1.38, respectively. At baseline mean z-score for FEV1 was -2.61±1.82 (SD). Over 2-years (n=59) mean FEV1 and FVC improved by 0.17 (p=0.039, 95% CI 0.01 to 0.34) and 0.21 (p=0.016, 95% CI 0.04 to 0.39) z-scores per annum, respectively. Over 4-years, there was improvement in height-for-age z-scores (slope 0.054, p=0.01, 95% CI 0.01 to 0.095) but no improvement in other anthropometric variables. There was no change in spirometry [FEV1 slope 0.00, p=0.999, 95% CI -0.09 to 0.09 and forced vital capacity (FVC) slope 0.09, p=0.859, 95% CI -0.09 to 0.1].

Conclusions: Children with non-CF bronchiectasis show an adequate growth over time; lung function stabilised but did not normalise with treatment, underscoring the need for early detection and institution of appropriate therapy.