Radiological and functional changes over 3 years in young children with cystic fibrosis

Area of Science:

• Pediatric Pulmonology
• Medical Imaging
• Respiratory Medicine

Background:

• Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
• Early detection and monitoring of airway disease progression in young children with CF are crucial for timely intervention.
• Assessing disease progression requires a combination of imaging and physiological measurements.

Purpose of the Study:

• To evaluate the progression of airway disease in young children with CF over a three-year period.
• To compare changes in chest radiology, expiratory interrupter resistance (Rint,exp), and spirometry.
• To identify potential subclinical signs of disease progression in preschool and school-aged children with CF.

Main Methods:

• A longitudinal study involving 21 preschool and 30 school-aged children with CF.
• Chest radiographs were taken at baseline and after three years, scored using five CF scoring systems.
• Expiratory interrupter resistance (Rint,exp) was measured at both time points; spirometry was assessed in school-aged children.

Main Results:

• Chest radiograph scores significantly worsened over the three-year study period, particularly in preschool children using the Wisconsin score.
• Expiratory interrupter resistance (Rint,exp) Z-scores remained largely within the normal range, with no significant annual changes.
• In school-aged children, only the forced expiratory volume in one second as a percentage of forced vital capacity showed a significant decline.

Conclusions:

• Chest radiography reveals significant worsening of airway disease in young children with CF over time.
• Lung function, as measured by Rint,exp and spirometry, appears stable in the short term despite radiological evidence of disease progression.
• Radiological changes may precede detectable functional impairment, highlighting the importance of imaging in monitoring early CF lung disease.