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Impact of CF on the Kidneys

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Cystic Fibrosis

Part of the book series: Respiratory Medicine ((RM))

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Abstract

For many years, the emphasis in CF care has been on the careful monitoring of nutrition and aggressive management of lung disease in order to improve the life span and quality of life of individuals with CF. However, as people with CF live longer, “hidden” complications of CF and CF therapies, such as renal disease, become increasingly important. While CFTR is expressed in the kidney, a direct renal phenotype of CFTR dysfunction has not been clearly defined. However, acute kidney injury, chronic kidney disease, and nephrolithiasis are all more commonly reported in CF compared to the general population, particularly post-lung transplant. Acute kidney injury typically relates to dehydration and aminoglycoside-related nephrotoxicity. For chronic kidney disease, CF-related diabetes requiring insulin is a significant risk factor both pre- and post-lung transplant. Other rarer causes of chronic kidney disease observed in CF relate to uncontrolled infection and inflammation including amyloid A (AA) amyloidosis and IgA nephropathy. A number of CF-related factors increase the risk of calcium oxalate kidney stones including the frequent use of antibiotics, malabsorption, and dehydration. Future studies need to focus on more reliable biomarkers to monitor renal function and detect renal damage in the CF population. Furthermore, preventative strategies are urgently required to mitigate the impact of CFRD on the risk of chronic kidney disease.

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Acknowledgments

We would like to acknowledge and thank Lori Van Balkom for sharing her experience living with chronic kidney disease post-lung transplantation. We would also like to thank Dr. Chris Flask and Dr. Kimberley McBennett from Case Western Reserve University and Dr. Tom McCulloch from Nottingham University Hospitals NHS Trust for their contributions.

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Authors and Affiliations

  1. The University of Nottingham, Child Health, Obstetrics and Gynaecology, Queens Medical Centre, Nottingham, UK

    Andrew Prayle

  2. St. Paul’s Hospital, University of British Columbia, Department of Medicine, Vancouver, BC, Canada

    Bradley S. Quon

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Correspondence to Bradley S. Quon .

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Editors and Affiliations

  1. The University of North Carolina at Chapel Hill, Department of Pediatrics, UNC Children’s Hospital, Chapel Hill, NC, USA

    Stephanie Duggins Davis

  2. Department of Pediatrics, University of Washington School of Medicine, Division of Pulmonary and Sleep Medicine, Seattle Children’s Hospital, Seattle, WA, USA

    Margaret Rosenfeld

  3. Department of Pediatrics, Indiana University School of Medicine, Division of Pediatric Pulmonology, Allergy and Sleep Medicine, Riley Hospital for Children at IU Health, Indianapolis, IN, USA

    James Chmiel

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Prayle, A., Quon, B.S. (2020). Impact of CF on the Kidneys. In: Davis, S., Rosenfeld, M., Chmiel, J. (eds) Cystic Fibrosis. Respiratory Medicine. Humana, Cham. https://doi.org/10.1007/978-3-030-42382-7_19

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  • DOI: https://doi.org/10.1007/978-3-030-42382-7_19

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