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

Autor: Patel N; Division of Pulmonary Disease, Critical Care and Sleep Medicine, University of Kansas Medical Center, Kansas City, Kansas, USA., Ansar M; Department of Pediatrics, University of North Carolina School of Medicine, Chapel Hill, North Carolina, USA., Pham A; Division of Pulmonary Disease, Critical Care and Sleep Medicine, University of Kansas Medical Center, Kansas City, Kansas, USA., Thomsen K; Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Vanderbilt University Medical Center, Nashville, Tennessee, USA., McKinzie CJ; Department of Pharmacy, University of North Carolina Medical Center, Chapel Hill, North Carolina, USA., Polineni D; Division of Allergy and Pulmonary Medicine, Washington University School of Medicine, St. Louis, Missouri, USA., Esther CR Jr; Division of Pediatric Pulmonology, Department of Pediatrics, University of North Carolina School of Medicine, Chapel Hill, North Carolina, USA., Brown RF; Department of Pediatrics, Division of Allergy, Immunology and Pulmonary Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Jazyk: angličtina
Zdroj: Pediatric pulmonology [Pediatr Pulmonol] 2024 Apr; Vol. 59 (4), pp. 863-866. Date of Electronic Publication: 2024 Jan 05.
DOI: 10.1002/ppul.26831
Abstrakt: 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.
(© 2024 Wiley Periodicals LLC.)
Databáze: MEDLINE
Externí odkaz: