Kidney disease is surprisingly common in People with Cystic Fibrosis (PwCF) but often recognized only after advanced damage has occurred. This irreversible chronic kidney disease (CKD) decreases both life expectancy and quality. The direct contribution of defective CFTR to CKD has been understudied, with recurrent exposure to medications commonly blamed. As novel medications stabilize lung disease in most PwCF, life-limiting manifestations of CFTR dysfunction in other organs merit further attention. The leader in CF research, CF Foundation (CFF) is currently funding a project by a research team with deep expertise in nephrology, immunology, and pulmonology probing the fundamental mechanisms underpinning kidney disease in PwCF. The study team is led by three Principal Investigators: a nephrologist from the University of Virginia, Charlottesville, VA (Dr. Swiatecka-Urban), pulmonologist from University of Alabama at Birmingham, Birmingham, AL (Harris), and an immunologist from Dartmouth-Hitchcock Medical Center, Geisel School of Medicine at Dartmouth, Hanover, NH (Dr. Skopelja-Gardner).
Additional resources:
| Lung-kidney axis in cystic fibrosis: Early urinary markers of kidney injury correlate with neutrophil activation and worse lung function.Rosner GM, Goswami HB, Sessions K, Mendyka LK, Kerin B, Vlasac I, Mellinger D, Gwilt L, Hampton TH, Graber M, Ashare A, Harris WT, Christensen B, Stanton BA, Swiatecka-Urban A, Skopelja-Gardner SJ Cyst Fibros. 2025 Jan 2; pii: S1569-1993(24)01858-7. doi: 10.1016/j.jcf.2024.12.007. Epub 2025 Jan 2.PMID: 39753455 |
For a deeper understanding of the extrapulmonary manifestations of cystic fibrosis, you might find the following presentation insightful:
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