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Gilbert’s Syndrome Leads to Elevated Bilirubin after Initiation of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis
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  • Nidhi Patel,
  • Maria Ansar,
  • Anh Pham,
  • Kelly Thomsen,
  • Cameron McKinzie,
  • Deepika Polineni,
  • Charles Esther,
  • Rebekah Brown
Nidhi Patel
no affiliation

Corresponding Author:[email protected]

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Maria Ansar
The University of North Carolina at Chapel Hill Department of Pediatrics
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Anh Pham
no affiliation
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Kelly Thomsen
Vanderbilt University Medical Center
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Cameron McKinzie
UNC Medical Center
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Deepika Polineni
Washington University in St Louis
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Charles Esther
The University of North Carolina at Chapel Hill School of Medicine
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Rebekah Brown
Vanderbilt University Medical Center
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Abstract

Nine people with cystic fibrosis (pwCF) were found to have isolated elevations in serum total bilirubin after starting elexacaftor/tezacaftor/ivacaftor (ETI) that were associated with Gilbert’s Syndrome. In longitudinal examination, total bilirubin levels increased substantially after initiation of ETI without elevations in liver transaminases in those with this syndrome. Because elevated bilirubin levels in Gilbert’s Syndrome are benign, ETI was able to be continued in these individuals. Genetic testing for this relatively common syndrome should be strongly considered for pwCF experiencing isolated hyperbilirubinemia after starting ETI, since appropriate diagnosis may help pwCF avoid unnecessary interruption in this therapy with significant health benefits in CF.
26 Jun 2023Submitted to Pediatric Pulmonology
27 Jun 2023Submission Checks Completed
27 Jun 2023Assigned to Editor
27 Jun 2023Review(s) Completed, Editorial Evaluation Pending
17 Jul 2023Reviewer(s) Assigned
19 Aug 2023Editorial Decision: Revise Major
17 Nov 20231st Revision Received
17 Nov 2023Submission Checks Completed
17 Nov 2023Assigned to Editor
17 Nov 2023Review(s) Completed, Editorial Evaluation Pending
17 Nov 2023Reviewer(s) Assigned