Journal of Pediatric Gastroenterology and Nutrition

Volume 71, Issue 4 pp. 421-422

Topic of the Month

Cystic Fibrosis-related Liver Disease

The Next Challenge

Robert D. Baker,

Corresponding Author

Robert D. Baker

[email protected]

Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, The State University of New York, NY

Address correspondence and reprint requests to Robert D. Baker, MD, PhD, Department of Pediatrics, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, The State University of New York, 39 Irving Place, Buffalo, NY 14201 (e-mail: [email protected]).Search for more papers by this author

Susan S. Baker,

Susan S. Baker

Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, The State University of New York, NY

Search for more papers by this author

Robert D. Baker,

Corresponding Author

Robert D. Baker

[email protected]

Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, The State University of New York, NY

Susan S. Baker,

Susan S. Baker

Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, The State University of New York, NY

Search for more papers by this author

First published: 30 July 2020

https://doi.org/10.1097/MPG.0000000000002867

Citations: 5

The authors report no conflicts of interest.

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ABSTRACT

Up to 40% of individuals with cystic fibrosis have cystic fibrosis-related liver disease (CFLD); however, only 5% to 10% will have clinically evident disease. With the introduction of powerful cystic fibrosis transmembrane conductance regulator (CFTR) enhancers, effective treatment for cystic fibrosis is available. The role of CFTR enhancers in liver disease is unknown at this time. The traditionally accepted theory of the pathogenesis of CFLD is being questioned. A different pathogenesis may lead to new ways to treat CFLD. The way that CFLD is diagnosed and monitored is evolving as new imaging technology become available.

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Volume71, Issue4

October 2020

Pages 421-422

Cystic Fibrosis-related Liver Disease

The Next Challenge

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The Next Challenge

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