Risk Factors for Bronchiectasis in Children With Cystic Fibrosis

Early Childhood Risk Factors for Decreased FEV1at Age 6-7 Years in Young Children with Cystic Fibrosis

“…Potential risk factors reported by parents on the annual family survey included maternal education, household income, immunizations, any exposure to environmental tobacco smoke, breastfeeding, wood-burning stoves, and swimming pools or hot tubs in the previous year. FEV 1 was expressed as a percentage of the predicted value for a healthy reference population based on the equations of Wang and colleagues (14). The data cutoff for the current analysis was December 31, 2013.…”

“…Children with cystic fibrosis (CF) can begin to develop lung disease within the first few months of life (1). However, there are limited objective measurements available to determine the severity of lung disease before children are reliably able to perform spirometry, generally at age 6 years (2).…”

Early lung surveillance of cystic fibrosis: what have we learnt?

“…Using this approach, AREST CF has elucidated that CF epithelium mounts an abnormal interferon and apoptotic response to rhinovirus [12]. A more recent study by AREST CF [13] demonstrated in vitro that NE levels observed in BAL are likely to prevent epithelial homeostasis and repair postinjury [3]. Future opportunities include combining bronchial brushing with conditional reprogramming, a technique of culturing epithelial cells with an irradiated fibroblast feeder stock that permits almost limitless generation of air-liquid interface models from patient's bronchial epithelial cells [14].…”

“…More sensitive metagenomic approaches that improve quantification of bacteria and viruses in BAL, combined with more detailed neutrophil characterization [11], will provide insight into initial pathogen exposures and the development of chronic neutrophilic inflammation in the CF airways. However, in light of the data demonstrating NE is a significant risk factor for bronchiectasis [3] and associated with dysregulated activation of MMPs [6], therapies targeted at controlling protease inflammation in early life may complement current antibacterial treatment protocols.…”

Imaging of Cystic Fibrosis Lung Disease and Clinical Interpretation

“…High-end CT scanners provide a high-pitch mode that delivers nearly artifact-free images even in free-breathing children without the need for sedation (• ▶ Table 1). To acquire paired inspiratory and expiratory scans in uncooperative children, usually anesthesia, intubation and controlled ventilation are necessary [25]. However, this would rather be applied for research than for clinical imaging.…”