Gilbert's syndrome leads to elevated bilirubin after initiation of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis

Author:

Patel Nidhi1ORCID,Ansar Maria2,Pham Anh1,Thomsen Kelly3,McKinzie Cameron J.4ORCID,Polineni Deepika5,Esther Charles R.6ORCID,Brown Rebekah F.7

Affiliation:

1. Division of Pulmonary Disease, Critical Care and Sleep Medicine University of Kansas Medical Center Kansas City Kansas USA

2. Department of Pediatrics University of North Carolina School of Medicine Chapel Hill North Carolina USA

3. Department of Pediatric Gastroenterology, Hepatology, and Nutrition Vanderbilt University Medical Center Nashville Tennessee USA

4. Department of Pharmacy University of North Carolina Medical Center Chapel Hill North Carolina USA

5. Division of Allergy and Pulmonary Medicine Washington University School of Medicine St. Louis Missouri USA

6. Division of Pediatric Pulmonology, Department of Pediatrics University of North Carolina School of Medicine Chapel Hill North Carolina USA

7. Department of Pediatrics, Division of Allergy, Immunology and Pulmonary Medicine Vanderbilt University Medical Center Nashville Tennessee USA

Abstract

AbstractNine 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.

Publisher

Wiley

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