Revue des maladies respiratoires · 2026
[Factors associated with FEV1 evolution in cystic fibrosis patients treated by CFTR modulator tritherapy].
Аннотация на английскомСтатьяНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Belard N, Blin T, De Luca A, Adam S Marchand, Plantier L, Flament Th, Mankikian J
Аннотация
INTRODUCTION: Triple therapy with Elexacaftor, Tezacaftor and Ivacaftor (ETI) has transformed the management of cystic fibrosis. The aim of this study was to describe the patients under treatment who do not show improvement in FEV1.
METHODS: In a single-center retrospective study of 63 adults, data were collected over the 12 months before and after initiation of ETI. Patients were classified as "improved FEV1" (FEV1 gain≥5% at 12 months of ETI) and "stable FEV1" (FEV1 gain<5%).
RESULTS: The patients in our study were at least 18 years old, suffered from cystic fibrosis, and had initiated a course of treatment by elexacaftor/tezacaftor/ivacaftor (ETI) between December 2019 and December 2021. They presented with either homozygosity or heterozygosity for the Phe508del mutation, with minimal function of the CFTR gene. At 12 months of ETI, an improvement in sweat chloride (-56 [-34; -69] mmol/L), FEV1 +17 [9; 24] % pred), RV/TLC ratio (-6 [-10; -3] %), walking distance +28 [14; 82] m and BMI (+1.5 [0.7; 2.6] kg/m2) was observed. Aside from the RV/TLC ratio, the improvement was similar between the "stable FEV1" (7 patients) and "improved FEV1" (56 patients) groups.
CONCLUSION: A benefit in terms of sweat chlorine, BMI, walking distance and exacerbations was observed in patients whose FEV1 remained stable on ETI. This observation underlines the importance of multiparametric evaluation of patients treated with CFTR modulators.
METHODS: In a single-center retrospective study of 63 adults, data were collected over the 12 months before and after initiation of ETI. Patients were classified as "improved FEV1" (FEV1 gain≥5% at 12 months of ETI) and "stable FEV1" (FEV1 gain<5%).
RESULTS: The patients in our study were at least 18 years old, suffered from cystic fibrosis, and had initiated a course of treatment by elexacaftor/tezacaftor/ivacaftor (ETI) between December 2019 and December 2021. They presented with either homozygosity or heterozygosity for the Phe508del mutation, with minimal function of the CFTR gene. At 12 months of ETI, an improvement in sweat chloride (-56 [-34; -69] mmol/L), FEV1 +17 [9; 24] % pred), RV/TLC ratio (-6 [-10; -3] %), walking distance +28 [14; 82] m and BMI (+1.5 [0.7; 2.6] kg/m2) was observed. Aside from the RV/TLC ratio, the improvement was similar between the "stable FEV1" (7 patients) and "improved FEV1" (56 patients) groups.
CONCLUSION: A benefit in terms of sweat chlorine, BMI, walking distance and exacerbations was observed in patients whose FEV1 remained stable on ETI. This observation underlines the importance of multiparametric evaluation of patients treated with CFTR modulators.