The use of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis postliver transplant: A case series.
Pediatr Pulmonol
; 57(2): 411-417, 2022 02.
Article
de En
| MEDLINE
| ID: mdl-34850610
ABSTRACT
INTRODUCTION:
Cystic fibrosis (CF)-related liver disease (CFLD) manifests as a wide spectrum of hepatobiliary disease and can progress to need liver transplantation. Elexacaftor/tezacaftor/ivacaftor (elx/tez/iva) is a cystic fibrosis transmembrane conductance regulator modulator that has superior efficacy compared to previously approved modulators. Use of elx/tez/iva, should be approached with caution in individuals with CFLD or following liver transplantation due to possible increases in liver function tests (LFTs) and drug-drug interactions with several immunosuppressant medications.OBJECTIVE:
The purpose of this case series is to explore if the use of elx/tez/iva is safe and tolerable in patients with CF postliver transplantation.METHODS:
A retrospective case series including patients prescribed elx/tez/iva following liver transplantation and an immunosuppressive regimen consisting of drug therapy metabolized by P-glycoprotein was completed.RESULTS:
Ten patients at six CF centers with a median age of 22.1 years (range 14-43.4 years) and the median time from the transplant of 6.9 years (range 0.6-22 years) were included. Most patients (8, 80%) received a reduced or full dose of elx/tez/iva for a mean duration of 10.4 months (range 7-12 months). Fluctuations in LFTs occurred in all patients (10, 100%) and led to therapy discontinuation in two patients (20%). Elx/tez/iva initiation resulted in elevations in tacrolimus trough concentration in seven patients (70%). Most patients who tolerated elx/tez/iva had symptomatic and quality of life improvement, increased body mass index, and maintained or improved lung function.CONCLUSION:
Initiation of elx/tez/iva in patients with CF who received liver transplantation may be safe with clinical benefits.Mots clés
Texte intégral:
1
Collection:
01-internacional
Base de données:
MEDLINE
Sujet principal:
Mucoviscidose
Type d'étude:
Observational_studies
/
Risk_factors_studies
Aspects:
Patient_preference
Limites:
Adolescent
/
Adult
/
Humans
Langue:
En
Journal:
Pediatr Pulmonol
Sujet du journal:
PEDIATRIA
Année:
2022
Type de document:
Article
Pays d'affiliation:
États-Unis d'Amérique