Gilbert's syndrome leads to elevated bilirubin after initiation of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis.
Pediatr Pulmonol
; 59(4): 863-866, 2024 Apr.
Article
em En
| MEDLINE
| ID: mdl-38179880
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.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Pirazóis
/
Piridinas
/
Pirrolidinas
/
Quinolonas
/
Fibrose Cística
/
Doença de Gilbert
/
Indóis
Limite:
Humans
Idioma:
En
Revista:
Pediatr Pulmonol
Assunto da revista:
PEDIATRIA
Ano de publicação:
2024
Tipo de documento:
Article
País de afiliação:
Estados Unidos