Kidney effects of triple CFTR modulator therapy in people with cystic fibrosis.

ABSTRACT:

Background

Elexacaftor/tezacaftor/ivacaftor (ETI) is a new cystic fibrosis transmembrane conductance regulator (CFTR) modulator that has transformed the respiratory prognosis of people with cystic fibrosis (pwCF). However, its impact on other organs such as the kidneys, where CFTR is expressed, remains unclear. Since pwCF are risk of both kidney disease and urolithiasis, we aimed to study the potential effects of ETI on renal function, volume status, and risk factors for urolithiasis.

Methods

This prospective, observational, single-center, before-after cohort study, involved adult pwCF eligible for ETI. The changes in plasma and urinary profiles were assessed by comparing renal function (using 2021 CKD-EPI_creatinine and 2021 CKD-EPI_{creatinine-cystatin C}

SUBMITTER: Gabai P 

PROVIDER: S-EPMC11443170 | biostudies-literature | 2024 Oct

REPOSITORIES: biostudies-literature