A screening tool to identify risk for bronchiectasis progression in children with cystic fibrosis
Pediatric Pulmonology, vol. 57, pp. 122–131
Abstract
BACKGROUND: The marked heterogeneity in cystic fibrosis (CF) disease complicates the selection of those most likely to benefit from existing or emergent treatments. OBJECTIVE: We aimed to predict the progression of bronchiectasis in preschool children with CF. METHODS: Using data collected up to 3 years of age, in the Australian Respiratory Early Surveillance Team for CF cohort study, clinical information, chest computed tomography (CT) scores, and biomarkers from bronchoalveolar lavage were assessed in a multivariable linear regression model as predictors for CT bronchiectasis at age 5-6. RESULTS: ) of the variance in bronchiectasis score. The strongest predictors of future bronchiectasis were: pancreatic insufficiency, repeated intravenous treatment courses, recurrent lower respiratory infections in the first 3 years of life, and lower airway inflammation. Dichotomizing the resulting prediction score at a bronchiectasis score of above the median resulted in a diagnostic odds ratio of 13 (95% confidence interval [CI], 6.3-27) with positive and negative predictive values of 80% (95% CI, 72%-86%) and 77% (95% CI, 69%-83%), respectively. CONCLUSION: Early assessment of bronchiectasis risk in children with CF is feasible with reasonable precision at a group level, which can assist in high-risk patient selection for interventional trials. The unexplained variability in disease progression at individual patient levels remains high, limiting the use of this model as a clinical prediction tool.
Authors 10
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Daan Caudri corresponding
The Kids Research Institute Australia · The University of Western Australia · Erasmus MC
Affiliation as printed
Department of Pediatrics/Respiratory Medicine Erasmus MC Rotterdam The Netherlands
Department of Respiratory Medicine Princess Margaret Hospital Perth Australia
Telethon Kids Institute The University of Western Australia Perth Australia
Department of Pediatrics/Respiratory Medicine, Erasmus MC, Rotterdam, The Netherlands
Department of Respiratory Medicine, Princess Margaret Hospital, Perth, Australia
Telethon Kids Institute, The University of Western Australia, Perth, Australia
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The Kids Research Institute Australia · The University of Western Australia
Affiliation as printed
Telethon Kids Institute The University of Western Australia Perth Australia
Telethon Kids Institute, The University of Western Australia, Perth, Australia
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The Kids Research Institute Australia · The University of Western Australia
Affiliation as printed
Telethon Kids Institute The University of Western Australia Perth Australia
Telethon Kids Institute, The University of Western Australia, Perth, Australia
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The Kids Research Institute Australia · The University of Western Australia
Affiliation as printed
Telethon Kids Institute The University of Western Australia Perth Australia
Telethon Kids Institute, The University of Western Australia, Perth, Australia
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Princess Margaret Hospital for Children
Affiliation as printed
Department of Diagnostic Imaging Princess Margaret Hospital for Children Perth Western Australia Australia
Department of Diagnostic Imaging, Princess Margaret Hospital for Children, Perth, Western Australia, Australia
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Ewout Willem Steyerberg Aachen Department of Medical Statistics and Bioinformatics Leiden University Medical Center Leiden The Netherlands Department of Medical Statistics and Bioinformatics
Leiden University Medical Center · Erasmus MC
Affiliation as printed
Department of Medical Statistics and Bioinformatics Leiden University Medical Center Leiden The Netherlands
Department of Public Health Erasmus MC Rotterdam The Netherlands
Department of Medical Statistics and Bioinformatics, Leiden University Medical Center, Leiden, The Netherlands
Department of Public Health, Erasmus MC, Rotterdam, The Netherlands
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Royal Children's Hospital · The University of Melbourne · Murdoch Children's Research Institute
Affiliation as printed
Department of Paediatrics University of Melbourne Parkville Australia
Department of Respiratory Medicine Royal Children's Hospital Parkville Australia
Murdoch Children's Research Institute Parkville Australia
Department of Paediatrics, University of Melbourne, Parkville, Australia
Department of Respiratory Medicine, Royal Children's Hospital, Parkville, Australia
Murdoch Children's Research Institute, Parkville, Australia
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Affiliation as printed
Child Health Research Centre The University of Queensland Brisbane Australia
Child Health Research Centre, The University of Queensland, Brisbane, Australia
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The Kids Research Institute Australia · The University of Western Australia
Affiliation as printed
Department of Respiratory Medicine Princess Margaret Hospital Perth Australia
Telethon Kids Institute The University of Western Australia Perth Australia
Department of Respiratory Medicine, Princess Margaret Hospital, Perth, Australia
Telethon Kids Institute, The University of Western Australia, Perth, Australia
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The Kids Research Institute Australia · The University of Western Australia · Hebrew University of Jerusalem · Hadassah Medical Center
Affiliation as printed
Department of Respiratory Medicine Princess Margaret Hospital Perth Australia
Pediatric Pulmonology and CF Unit, Department of Pediatrics Hadassah‐Hebrew University Medical Center Jerusalem Israel
Telethon Kids Institute The University of Western Australia Perth Australia
Department of Respiratory Medicine, Princess Margaret Hospital, Perth, Australia
Pediatric Pulmonology and CF Unit, Department of Pediatrics, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
Telethon Kids Institute, The University of Western Australia, Perth, Australia
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