Granados Andrea, Chan Christine L, Moheet Amir, Vigers Timothy, Arbeláez Ana María, Larson Ode Katie
Department of Pediatrics, Nicklaus Children's Hospital, Miami, Florida, USA.
Department of Pediatrics, Children's Hospital Colorado, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA.
Pediatr Pulmonol. 2023 Jun;58(6):1805-1811. doi: 10.1002/ppul.26388. Epub 2023 Mar 17.
The effects of elexacaftor-tezacaftor-ivacaftor (ETI) on body composition in people with CF (pwCF) are unknown.
Dual-energy X-ray absorptiometry fat-free mass and fat mass adjusted for height (FMI) as well as oral glucose tolerance test derived measures of insulin secretion and sensitivity were compared before and after ETI initiation in eight pwCF.
Patients median age: 22 years interquartile range (IQR: 16-28), 87.5% male, median time on ETI:11 months. Weight z-score increased from -0.52 to 0.18 (p = 0.014); FMI increased from 4.12 to 6.29 (p = 0.014). Insulin secretion (C pep iAUC/Gluc iAUC) increased from 8.71 to 14.21 (p = 0.021), insulin resistance (HOMA2 IR) increased from 0.73 to 1.25 (p = 0.014) and insulin sensitivity decreased (Matsuda) 8.88 to 5.58 (p = 0.036).
ETI resulted in increased weight and fat mass. BMI and muscle mass did not change. Both insulin secretion and insulin resistance increased. Longer-term metabolic consequences of ETI need further investigation.
依列卡福妥-替扎卡福妥-依伐卡福妥（ETI）对囊性纤维化患者（pwCF）身体成分的影响尚不清楚。
对8例pwCF患者在开始使用ETI之前和之后，比较了双能X线吸收法测量的去脂体重和根据身高调整的脂肪量指数（FMI），以及口服葡萄糖耐量试验得出的胰岛素分泌和敏感性指标。
患者中位年龄：22岁，四分位间距（IQR：16 - 28），87.5%为男性，ETI治疗的中位时间：11个月。体重Z评分从 -0.52增加至0.18（p = 0.014）；FMI从4.12增加至6.29（p = 0.014）。胰岛素分泌（C肽iAUC/葡萄糖iAUC）从8.71增加至14.
