Longitudinal growth and lung function in pediatric non-cystic fibrosis bronchiectasis: what influences lung function stability?

Abstract

Background: Longitudinal FEV(1) data in children with non-cystic fibrosis (non-CF) bronchiectasis (BE) are contradictory, and there are no multifactor data on the evolution of lung function and growth in this group. We longitudinally reviewed lung function and growth in children with non-CF BE and explored biologically plausible factors associated with changes in these parameters over time.

Methods: Fifty-two children with > or = 3 years of lung function data were retrospectively reviewed. Changes in annual anthropometry and spirometry at year 3 and year 5 from baseline were analyzed. The impact of sex, age, cause, baseline FEV(1), exacerbation frequency, radiologic extent, socioeconomic status, environmental tobacco smoke exposure, and period of diagnosis was evaluated.

Results: Over 3 years, the group mean forced expiratory flow midexpiratory phase percent predicted and BMI z-score improved by 3.01 (P = .04; 95% CI, 0.14-5.86) and 0.089 (P = .01; 95% CI, 0.02-0.15) per annum, respectively. FEV(1)% predicted, FVC% predicted, and height z-score all showed nonsignificant improvement. Over 5 years, there was improvement in FVC% predicted (slope 1.74; P = .001) annually, but only minor improvement in other parameters. Children with immunodeficiency and those with low baseline FEV(1) had significantly lower BMI at diagnosis. Frequency of hospitalized exacerbation and low baseline FEV(1) were the only significant predictors of change in FEV(1) over 3 years. Decline in FEV(1)% predicted was large (but nonsignificant) for each additional year in age of diagnosis.

Conclusions: Spirometric and anthropometric parameters in children with non-CF BE remain stable over a 3- to 5-year follow-up period once appropriate therapy is instituted. Severe exacerbations result in accelerated lung function decline. Increased medical cognizance of children with chronic moist cough is needed for early diagnosis, better management, and improving overall outcome in BE.