在患有囊性纤维化的年轻成年人中，接受依列卡福妥-替扎卡福妥-依伐卡福妥治疗后脂肪量增加及肥胖风险上升。

Increased fat mass and obesity risk after elexacaftor-tezacaftor-ivacaftor therapy in young adults with cystic fibrosis.

作者信息

Merino Sánchez-Cañete Ana, López Cárdenes Concepción Marina, Vicente Santamaría Saioa, Gutiérrez Martínez José Ramón, Suárez González Marta, Álvarez Merino María, González Jiménez David

机构信息

Cystic Fibrosis Unit, Department of Pediatrics, University Hospital Ramón y Cajal, Madrid, Spain.

Cystic Fibrosis Unit, University Hospital Central de Asturias, Oviedo, Spain.

出版信息

Front Nutr. 2024 Nov 7;11:1477674. doi: 10.3389/fnut.2024.1477674. eCollection 2024.

DOI:10.3389/fnut.2024.1477674

PMID:39582664

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11582979/

Abstract

BACKGROUND

When people with cystic fibrosis (PwCFs) are treated with cystic fibrosis transmembrane conductance regulator protein modulator (CFTRm), it leads to changes in body composition. An easy, non-invasive, and reproducible method to assess this is by means of electrical bioimpedance measurement, which complements the information provided by the already-known anthropometric parameters.

METHODS

Seventeen adults with cystic fibrosis in treatment with elexacaftor-tezacaftor-ivacaftor (ETI) were recruited for a prospective, longitudinal, single-center study over 12 months. Study variables included weight, body mass index (BMI), and body composition by bioelectrical impedance analysis (BIA) [including fat mass (FM) and fat-free mass (FFM)].

RESULTS

At 12 months, there was an increase in overweight patients (5.9% vs. 23.5%) and a statistically significant increase in BMI at 6 and 12 months. An increase in FM and FFM was observed at 12 months. The increase was greater in FM (95% CI) from a baseline of 1.77% (0.00-3.54; = 0.050) at 6 months and 2.64% (0.48-4.81; = 0.020) at 12 months.

CONCLUSION

After treatment with ETI, there was an increase in weight, BMI, and FM more than FFM in PwCF. These changes would be confirmed in long-term studies to improve nutritional management.

摘要

背景

囊性纤维化患者（PwCFs）接受囊性纤维化跨膜传导调节蛋白调节剂（CFTRm）治疗时，身体成分会发生变化。一种简单、无创且可重复的评估方法是通过生物电阻抗测量，它补充了已知人体测量参数所提供的信息。

方法

招募了17名接受依列卡福-替扎卡福-依伐卡福（ETI）治疗的成年囊性纤维化患者，进行为期12个月的前瞻性、纵向、单中心研究。研究变量包括体重、体重指数（BMI）以及通过生物电阻抗分析（BIA）得出的身体成分[包括脂肪量（FM）和去脂体重（FFM）]。

结果

在12个月时，超重患者有所增加（5.9%对23.5%），且在6个月和12个月时BMI有统计学意义的显著增加。在12个月时观察到FM和FFM均增加。FM的增加幅度更大（95%CI），6个月时从基线的1.77%（0.00 - 3.54；P = 0.050）增加到12个月时的2.64%（0.48 - 4.81；P = 0.020）。