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A recent study of cystic fibrosis (CF) infants and toddlers, conducted by a multinational team of researchers including several from the Center for Cystic Fibrosis and Airways Disease Research (CF-AIR), has revealed potential metabolite biomarkers for monitoring and evaluating risk of lung disease in early life CF.
“Early life elevations of methionine oxidation and ornithine track and predict cystic fibrosis structural lung disease" was published in European Respiratory Journal Open Research on March 25, 2025, with Joshua Chandler, PhD, an Emory University Assistant Professor and the incoming Scientific Director of the Pediatric Metabolomics and Biomarkers Core (PMBC), as the corresponding author. The study identified metabolites in CF bronchoalveolar lavage (BAL) fluid associated with lung inflammation and lung structural damage. Ultimately, the researchers discovered that methionine sulfoxide, N-acetylmethionine, and ornithine could predict future bronchiectasis in CF toddlers, and could do so more sensitively than established biomarkers such as neutrophil elastase and interleukin-8.
“We hope measurement of these metabolites in early life could help pediatricians better gauge lung disease risk in their patients,” said Dr. Chandler. “They may also be useful for evaluating interventions targeting lung disease. To facilitate that, we are working on validating their utility in less invasive sampling methods, such as breath.”
An associate editor wrote of the article: “I anticipate and hope that your paper will serve as a guiding light in the evolving landscape of cystic fibrosis illuminating paths that will help rewrite the history around disease progression.”
Dr. Chandler cited crucial involvement of colleagues Rabindra Tirouvanziam, PhD and Lokesh Guglani, MD, whose IMPEDE-CF cohort forms a critical pillar of the publication, and Limin Peng, PhD, of the Rollins School of Public Health, for providing essential biostatistical guidance. Dr. Chandler also cited international and U.S.-based colleagues without whom the study would have been impossible, including researchers at the Erasmus MC-Sophia Children’s Hospital (Rotterdam, The Netherlands), the University of Melbourne and Royal Children’s Hospital (Melbourne, Australia), and the Telethon Kids Institute (Perth, Australia), and the University of North Carolina Chapel Hill.
The study’s first author was Emory graduate student Sarah Mansour, PhD, who successfully defended her thesis on April 15, 2025. Other Emory Department of Pediatrics contributors included Lisa Slimmen, MD (who was affiliated with the Erasmus MC when conducting this study), Lucas Silva, BS, Genoah Collins, MS, James Lyles, PhD, Vincent Giacalone, PhD, Camilla Margaroli, PhD, and Diego Moncada-Giraldo, PhD.
This study was funded by the National Institutes of Health, the Cystic Fibrosis Foundation, the Hertz Family Foundation, the Australian National Health and Medical Research Council, and the American Society of Hematology.
ERJ Open Research is an open access, original research journal published by the European Respiratory Society (ERS). A pre-proof version of the journal article can be found here.