Evidence map›Paper›PMID 40525743›Full record

ArticlePediatric pulmonology2025

Impact of Elexacaftor/Tezacaftor/Ivacaftor on Fecal Elastase-1 in Children With Cystic Fibrosis.

Senthilkumar Sankararaman, Leesa Prunty, Catherine Pamer, Aravind Thavamani, Erica Roesch, Teresa Schindler

Abstract read
In one paragraph

Article in Pediatric pulmonology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

4 citing papers in PubMed.

  1. Article
  2. Article
  3. Article
  4. The impact of elexacaftor-tezacaftor-ivacaftor on cardiometabolic risk factors: a systematic review.European respiratory review : an official journal of the European Respiratory Society · 2026
    Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

6 authors.

Senthilkumar SankararamanDivision of Pediatric Gastroenterology, Hepatology, and Nutrition, Cleveland Clinic Children's Hospital, Cleveland, Ohio, USA.
Leesa PruntyDivision of Pediatric Pulmonology, UH Rainbow Babies & Children's Hospital, Cleveland, Ohio, USA.
Catherine PamerDivision of Pediatric Pulmonology, UH Rainbow Babies & Children's Hospital, Cleveland, Ohio, USA.
Aravind ThavamaniDivision of Pediatric Gastroenterology, Hepatology, and Nutrition, UH Rainbow Babies & Children's Hospital, Cleveland, Ohio, USA.
Erica RoeschDivision of Pediatric Pulmonology, UH Rainbow Babies & Children's Hospital, Cleveland, Ohio, USA.
Teresa SchindlerDivision of Pediatric Pulmonology, UH Rainbow Babies & Children's Hospital, Cleveland, Ohio, USA.

Funding

The authors received no specific funding for this work.
6 · The paper itself

Abstract

backgroundPrior studies have documented improvement in pancreatic status after ivacaftor and lumacaftor/ivacaftor in a small number of patients with cystic fibrosis (CF). There is paucity of data with similar improvement after initiation of elexacaftor/tezacaftor/ivacaftor (ETI).

methodsIn this cross-sectional study, we retrospectively reviewed the change in fecal elastase-1 (FE-1) at least 6 months after initiation of ETI in children less than 12 years of age. Children who demonstrated a change in FE-1 of ≥ 100 µg/g from the baseline after ETI therapy (post-ETI FE-1 minus pre-ETI FE-1) were termed as responders and those with a change in FE-1 < 100 µg/g post-ETI (or no change in FE-1) were termed as nonresponders.

resultsThirty-five children had post-ETI FE-1 value at least 6 months after its initiation and were included for final review. The mean change (±SD) in FE-1 post-ETI from the baseline in the entire cohort was 47.2 ± 89.5. Seven children had a change in FE-1 of ≥ 100 µg/g or more post-ETI (responders). Two of these seven patients had a change in FE-1 of ≥ 200 µg/g post-ETI. Twenty-three had no change or change < 100 µg/g post-ETI (nonresponders). Another five children who did not have a baseline FE-1 and had post-ETI FE-1 < 10 µg/g were also added to the nonresponders cohort increasing the total number to 28. The pre- and post-ETI body mass index was significantly higher in the responder group compared to the nonresponders.

conclusionIn our cohort of 35 children, 7 (20%) were noted to have a positive change in FE-1 from the baseline value of ≥ 100 µg/g post-ETI. Further prospective multicenter studies will help identify predictive factors associated with improvement in FE-1 after initiation of CFTR-directed modulator therapies.

Indexed as

AminophenolsBenzodioxolesCystic FibrosisFecesIndolesPancreatic ElastasePyrazolesPyridinesPyrrolidinesQuinolonesChildChild, PreschoolChloride Channel AgonistsCross-Sectional StudiesDrug CombinationsFemaleAminophenolsBenzodioxolesChloride Channel AgonistsDrug Combinationselexacaftorelexacaftor, ivacaftor, tezacaftor drug combinationIndolesivacaftorPancreatic ElastasePyrazolesPyridinesPyrrolidinesQuinolinesQuinolonestezacaftortezacaftor, ivacaftor drug combinationCFCFTRcystic fibrosiselexacaftor/tezacaftor/ivacaftorfecal elastaseivacaftorlumacaftor/ivacaftormodulatorspancreatic function

Identifiers

PMID40525743
PMCPMC12172394

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.