P319 Effects of elexacaftor/tezacaftor/ivacaftor (ETI) on nutrition parameters and resting energy expenditure (REE) in people with cystic fibrosis (pwCF)

Cystic fibrosis (CF) is associated with increased energy requirement, loss and inadequate intake. With the approval of ETI, a highly effective CFTR-modulator therapy became available for 90% pwCF. First studies have shown promising improvements in anthropometric parameters. We investigated short-term change nutritional status under ETI potential mechanisms bodyweight (bw) gain. The prospective cohort study includes 26 pwCF pancreatic insufficiency, 56.8% F508del homozygous, median age 19 (12–32) years. Body mass index (BMI); fat (FM) fat-free (FFM) via bioelectrical impedance analysis; fecal calprotectin (FC); forced expiratory volume 1 s (FEV1%pred) spirometry REE indirect calorimetry were examined before(V1), ten (V2) 18 weeks (V3) after treatment. Dietary intake abdominal symptoms V1 V2 by 4-day food diary CF-specific questionnaire. Besides FEV1%pred (17,7 ± 12.2%), impressive improvement status, without intake, was observed during 18-week follow-up. A mean gain 4.4 2.8 kg bw, increases 2.5 FFM 1.6 1.5 FM observed. Mean increase BMI Z-Score 1.4 0.9 kg/m2 0.42 0.33. This can be rated as particularly positive variation explained FFM-index. Underweight decreased from 8/26 to 4/25; two patients developed overweight. After low transient weeks, REE%pred significantly 119.6 14.2% (V1) 106.4 23.6% (V3). Intestinal inflammation decreased. shows bw gain, probably underlying synergistic effect, decrease REE%pred. Current nutrition should reevaluated due growing incidence overweight ETI.