Introduction/Aim. To our knowledge, only one, small prospective longitudinal study of children with chronic suppurative lung disease (CSLD)/bronchiectasis has been published in the current era. We sought to extend this study by evaluating the longer-term clinical and lung function outcomes and their associated risk factors in Indigenous children and adolescents from Australia, Alaska and New Zealand who participated in our previous CSLD/bronchiectasis studies during 2004-2010.
Methods. Between 2015-2018, we evaluated 131/180 (72.8%) children and adolescents from the original studies at a single follow-up visit. We conducted standardised questionnaires, clinical examination and spirometry, and reviewed medical records and scored available chest computed-tomography scans.
Results. Participants were seen at a mean age of 12.3 years (standard deviation 2.6), a median of 9.0 years (range 5.0-13.0) after their original recruitment. With increasing age, rates of acute lower respiratory infections (ALRI) declined, while lung function was mostly within population norms (median forced expiry volume in one-second [FEV1] was 90% predicted, interquartile range [IQR] 81-105; forced vital capacity [FVC] of 98% predicted, IQR 85-114). However, 43/11 (38.7%) reported chronic cough episodes. Their overall global rating judged by symptoms, including ALRI frequency, in last 12 months, examination findings and spirometry (when available) was well (25/123; 20.3%), stable (54/123; 43.9%) or improved (44/123; 35.8%). Multivariable regression models found household tobacco exposure and age at first ALRI-episode as independent risk factors associated with lower FVC %predicted values.
Conclusion. With clinical care, the respiratory outcomes in late childhood/early adolescence are encouraging for these patient populations at high-risk of premature mortality. Prospective studies to further inform management throughout the life course are now needed.