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Increased Fat Mass and Obesity Risk After Elexacaftor-tezacaftor-ivacaftor Therapy in Young Adults with Cystic Fibrosis

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.

References
1.
Harris P, Taylor R, Thielke R, Payne J, Gonzalez N, Conde J . Research electronic data capture (REDCap)--a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform. 2008; 42(2):377-81. PMC: 2700030. DOI: 10.1016/j.jbi.2008.08.010. View

2.
Kuriyan R . Body composition techniques. Indian J Med Res. 2019; 148(5):648-658. PMC: 6366261. DOI: 10.4103/ijmr.IJMR_1777_18. View

3.
Goetz D, Savant A . Review of CFTR modulators 2020. Pediatr Pulmonol. 2021; 56(12):3595-3606. DOI: 10.1002/ppul.25627. View

4.
Proud D, Duckers J . Weight a minute: Exploring the effect on weight and body composition after the initiation of elexacaftor/tezacaftor/ivacaftor in adults with CF. J Cyst Fibros. 2023; 22(5):847-850. DOI: 10.1016/j.jcf.2023.06.002. View

5.
King S, Tierney A, Edgeworth D, Keating D, Williams E, Kotsimbos T . Body composition and weight changes after ivacaftor treatment in adults with cystic fibrosis carrying the G551 D cystic fibrosis transmembrane conductance regulator mutation: A double-blind, placebo-controlled, randomized, crossover study with.... Nutrition. 2021; 85:111124. DOI: 10.1016/j.nut.2020.111124. View