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PRAGMA-CF. A Quantitative Structural Lung Disease Computed Tomography Outcome in Young Children with Cystic Fibrosis
Tim Rosenow1, Merel C J Oudraad, Conor P Murray
11 School of Paediatrics and Child Health and.
Insights
A new quantitative method, Perth-Rotterdam Annotated Grid Morphometric Analysis for CF (PRAGMA-CF), accurately measures cystic fibrosis (CF) airway disease in young children. This sensitive and reproducible tool aids in assessing lung disease extent in pediatric CF patients.
Area of Science:
- Pediatric Pulmonology
- Radiology
- Medical Imaging Analysis
Background:
- Chest computed tomography (CT) is the standard for assessing cystic fibrosis (CF) airway disease.
- Current outcome measures lack standardization for children under 6 years old.
Purpose of the Study:
- To develop and validate the Perth-Rotterdam Annotated Grid Morphometric Analysis for CF (PRAGMA-CF) as a quantitative measure for pediatric CF airway disease.
- To compare PRAGMA-CF with the existing CF-CT scoring method.
Main Methods:
- PRAGMA-CF was applied to CT scans from the Australian Respiratory Early Surveillance Team for CF (AREST CF) cohort.
- The method involved annotating a grid on axial slices to quantify bronchiectasis, mucous plugging, and trapped air.
- Observer reliability and comparison with a validated standard were assessed using 30 scans each.
Main Results:
- PRAGMA-CF demonstrated high intraobserver reliability for %Dis (0.93), %Bx (0.93), and %TA (0.96).
- Changes in %Dis and %Bx measured by PRAGMA-CF correlated with neutrophil elastase presence at age 3.
- CF-CT showed a correlation only for bronchiectasis score with neutrophil elastase.
Conclusions:
- PRAGMA-CF is a sensitive and reproducible outcome measure for evaluating lung disease in very young children with CF.
- This quantitative method offers a standardized approach for assessing airway disease in pediatric CF patients.
Rationale:
Chest computed tomography (CT) is the gold standard for demonstrating cystic fibrosis (CF) airway disease. However, there are no standardized outcome measures appropriate for children younger than 6 years.
Objectives:
We developed the Perth-Rotterdam Annotated Grid Morphometric Analysis for CF (PRAGMA-CF), a quantitative measure of airway disease, and compared it with the commonly used CF-CT scoring method.
Methods:
CT scans from the Australian Respiratory Early Surveillance Team for CF (AREST CF) cohort in Western Australia were included. PRAGMA-CF was performed by annotating a grid overlaid on 10 axial slices for the presence of bronchiectasis, mucous plugging, or other airway abnormalities (inspiratory scans) and trapped air (expiratory scans). The separate proportions of total disease (%Dis), bronchiectasis (%Bx), and trapped air (%TA) were determined. Thirty scans were used for observer reliability, and 30 paired scans obtained at 1 and 3 years old were used for comparison with a validated standard and biologic plausibility.
Measurements And Main Results:
Intraobserver, intraclass correlation coefficients (95% confidence interval) for %Dis, %Bx, and %TA were 0.93 (0.86-0.97), 0.93 (0.85-0.96), and 0.96 (0.91-0.98), respectively. The change in %Dis (P = 0.004) and %Bx (P = 0.001) with PRAGMA-CF was related to neutrophil elastase presence at age 3, whereas only the change in bronchiectasis score was related to neutrophil elastase (P < 0.001) with CF-CT. Sample-size calculations for various effect sizes are presented.
Conclusions:
PRAGMA-CF is a sensitive and reproducible outcome measure for assessing the extent of lung disease in very young children with CF.
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