Improved Nutritional Outcomes and Gastrointestinal Symptoms in Adult Cystic Fibrosis Patients Treated with Elexacaftor/Tezacaftor/Ivacaftor

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00209805%3A_____%2F24%3A00079826" target="_blank" >RIV/00209805:_____/24:00079826 - isvavai.cz</a>

Alternative codes found

RIV/00216224:14110/24:00135924 RIV/00216208:11130/24:10481140 RIV/61989592:15110/24:73625807 RIV/65269705:_____/24:00079880 and 2 more

Result on the web

<a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11250406/" target="_blank" >https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11250406/</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1159/000538606" target="_blank" >10.1159/000538606</a>

Result language

angličtina

Original language name

Improved Nutritional Outcomes and Gastrointestinal Symptoms in Adult Cystic Fibrosis Patients Treated with Elexacaftor/Tezacaftor/Ivacaftor

Original language description

Introduction: Cystic fibrosis transmembrane conductance regulator modulator therapy improves nutritional status and quality of life. Clinical trials have shown pancreatic insufficiency conversion, mostly in pediatric patients treated with ivacaftor. Studies with elexacaftor/tezacaftor/ivacaftor (ETI) in older patients have not suggested restoration of exocrine pancreas function, but quality data in adults are lacking. Our aim was to show the effect of ETI in adults with cystic fibrosis (CF) on nutritional status and digestive function. We hypothesized improvement of nutritional parameters and gastrointestinal symptoms, reduction of pancreatic enzyme replacement therapy, but uncertain improvement in exocrine pancreatic function. Methods: We prospectively enrolled adults with CF treated with ETI from August 2021 to June 2022. We measured anthropometric parameters, laboratory nutritional markers, change of fecal elastase, pancreatic enzymes replacement therapy needs, and gastrointestinal symptoms. Results: In the cohort of 29 patients (mean age 29.1 years), 82.8% suffered exocrine pancreatic insufficiency. After ETI, mean BMI increased by 1.20 kg/m(2) (p &lt; 0.001), mean body weight by 3.51 kg (p &lt; 0.001), albumin by 2.81 g/L, and prealbumin by 0.06 (both p &lt; 0.001). Only 1 patient, initially pancreatic insufficient (4.5%, p &lt; 0.001), developed pancreatic sufficiency, indicated by increased fecal elastase from 45 mu g/g to 442.1 mu g/g. Mean change in lipase substitution decreased by 1,969 units/kg/day (p &lt; 0.001) and stools frequency by 1.18 per day (p &lt; 0.001). Conclusion: Our data suggest increased nutritional parameters, lower pancreatic substitution requirements, and improved defecation in adult CF patients on ETI. Improvement in exocrine pancreatic function might be mutation-specific and needs further study.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

—

OECD FORD branch

30219 - Gastroenterology and hepatology

Project

—

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2024

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Digestive diseases

ISSN

0257-2753

e-ISSN

1421-9875

Volume of the periodical

42

Issue of the periodical within the volume

4

Country of publishing house

DE - GERMANY

Number of pages

8

Pages from-to

361-368

UT code for WoS article

001243080000001

EID of the result in the Scopus database

2-s2.0-85195096508