Decoding liver injury in cystic fibrosis: How to tell drug-induced liver injury from cystic fibrosis liver disease

doi:10.3748/wjg.v32.i10.114946

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World Journal of Gastroenterology

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1007-9327

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Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA

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©Author(s) (or their employer(s)) 2026. No commercial re-use. See Permissions. Published by Baishideng Publishing Group Inc.

World J Gastroenterol. Mar 14, 2026; 32(10): 114946
Published online Mar 14, 2026. doi: 10.3748/wjg.v32.i10.114946

Decoding liver injury in cystic fibrosis: How to tell drug-induced liver injury from cystic fibrosis liver disease

Junseo Lee, Anuroop Yekula, Ava Wexler, William Zhuang, Ashwath Elangovan, Joshua Rosario, Philomena Burger, Gopal Ramaraju, Benyam Addissie, Nicholas Lim, Michael R Narkewicz, Patrick Twohig

Junseo Lee, Anuroop Yekula, Patrick Twohig, Department of Gastroenterology and Hepatology, University of Rochester Medical Center, Rochester, NY 14682, United States

Ava Wexler, Department of Internal Medicine, University of Rochester Medical Center, Rochester, NY 14682, United States

William Zhuang, Ashwath Elangovan, Joshua Rosario, Philomena Burger, Department of Medicine, University of Rochester Medical Center, Rochester, NY 14682, United States

Gopal Ramaraju, Benyam Addissie, Nicholas Lim, Department of Transplant Hepatology, University of Rochester Medical Center, Rochester, NY 14682, United States

Michael R Narkewicz, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO 80045, United States

Author contributions: Twohig P designed the overall concept and outline of the manuscript; Lee J, Yekula A, Wexler A, Zhuang W, Elangovan A, Rosario J, Burger P, and Twohig P conducted the literature review and drafted the manuscript; Lee J, Yekula A, Narkewicz MR, Ramaraju G, Lim N, Addissie B, and Twohig P made critical revisions; all authors approved the final version to publish.

Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.

PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.

Corresponding author: Patrick Twohig, MD, Assistant Professor, FRCPC, Department of Gastroenterology and Hepatology, University of Rochester Medical Center, 601 Elmwood Avenue, Rochester, NY 14682, United States. patrick_twohig@urmc.rochester.edu

Received: October 11, 2025
Revised: December 11, 2025
Accepted: January 8, 2026
Published online: March 14, 2026
Processing time: 151 Days and 13.6 Hours

Core Tip

Core Tip: Cystic fibrosis (CF) liver disease is a major comorbidity in CF, complicated by progressive cholestasis and overlapping risk of drug-induced liver injury, including from CF transmembrane conductance regulator modulators. This study synthesizes literature on pathogenesis, clinical features, diagnostics, and management to aid in distinguishing advanced CF liver disease progression from drug induced liver injury. Key differentiators include biochemical profiles, imaging, treatment response, and histology. Early recognition and a multidisciplinary approach are essential to optimize therapy, preserve liver function, and balance the benefits and risks of CF transmembrane conductance regulator modulators.