Evidence map›Paper›PMID 35474016›Full record

ArticleJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2022

Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients.

Kathleen J Ramos, Jennifer S Guimbellot, Maryam Valapour, Lauren E Bartlett, Travis Hee Wai, Christopher H Goss, Joseph M Pilewski, Albert Faro, Joshua M Diamond, CFLTC Study Group

Open access · greenAbstract read
In one paragraph

Article in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
14citing papers in PubMed, 1 pooled it
6.2field-weighted citation impact, top 3% of its field
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

14 citing papers in PubMed, 1 synthesis or guideline pooled it, 43 citations in OpenAlex.

  1. Pooled it
  2. 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
  3. Elexacaftor/tezacaftor/ivacaftor prescription in lung transplant recipients with cystic fibrosis in the US.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025
    Article
  4. Elexacaftor-tezacaftor-ivacaftor pharmacokinetics with concurrent tacrolimus administration after lung transplant.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025
    Observational
  5. Article
  6. Article
  7. The ageing of people living with cystic fibrosis: what to expect now?European respiratory review : an official journal of the European Respiratory Society · 2024
    Review
  8. Cystic fibrosis.Nature reviews. Disease primers · 2024
    Review
  9. Patient perspectives on elexacaftor/tezacaftor/ivacaftor after lung transplant.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2024
    Article
  10. Review
  11. Article
  12. Review
  13. A year in review (2022): Modulators and COVID19, the story goes on….Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2023
    Review
  14. Review
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

10 authors at 6 institutions in 1 country.

Kathleen J RamosDepartment of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of Washington, 1959 NE Pacific Street, Box 356522, Seattle, WA 98195, USA. Electronic address: ramoskj@uw.edu.
Jennifer S GuimbellotDepartment of Pediatrics, Division of Pulmonary and Sleep Medicine, University of Alabama at Birmingham, Birmingham, AL, USA.
Maryam ValapourRespiratory Institute, Cleveland Clinic, Cleveland, OH, USA.
Lauren E BartlettDepartment of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of Washington, 1959 NE Pacific Street, Box 356522, Seattle, WA 98195, USA.
Travis Hee WaiDepartment of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of Washington, 1959 NE Pacific Street, Box 356522, Seattle, WA 98195, USA.
Christopher H GossDepartment of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of Washington, 1959 NE Pacific Street, Box 356522, Seattle, WA 98195, USA; Department of Pediatrics, Division of Pulmonary and Sleep Medicine, University of Washington, Seattle, WA, USA.
Joseph M PilewskiDepartment of Medicine, Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA, USA.
Albert FaroCystic Fibrosis Foundation, Bethesda, MD, USA.
Joshua M DiamondDepartment of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
CFLTC Study Group
University of Washington · USCleveland Clinic · USCystic Fibrosis Foundation · USUniversity of Alabama at Birmingham · USUniversity of Pennsylvania · USUniversity of Pittsburgh · US

Funding

UAB CF Research and Translation Core CenterP30DK072482 · NIDDK · UNIVERSITY OF ALABAMA AT BIRMINGHAM · PI Brian R. Davis · 2007 to 2026
$23.0M
Translational Research Center to Expedite Novel Therapies in Cystic FibrosisP30DK089507 · NIDDK · SEATTLE CHILDREN'S HOSPITAL · PI Christopher Hooper Goss, Lucas R Hoffman · 2010 to 2026
$21.4M
Predicting survival and planning for lung transplantation in severe cystic fibrosisK23HL138154 · NHLBI · UNIVERSITY OF WASHINGTON · PI RAMOS, KATHLEEN JESSICA · 2017 to 2021
$947k
Pharmacometric approaches to precision optimization of ivacaftor response in cystic fibrosis patientsK23HL143167 · NHLBI · UNIVERSITY OF ALABAMA AT BIRMINGHAM · PI GUIMBELLOT, JENNIFER S · 2020 to 2024
$839k
NHLBI NIH HHS K23 HL138154NHLBI NIH HHS K23 HL143167NIDDK NIH HHS P30 DK072482NIDDK NIH HHS P30 DK089507
6 · The paper itself

Abstract

backgroundCystic fibrosis (CF) lung transplant (LT) recipients may warrant treatment with elexacaftor/tezacaftor/ivacaftor (ETI) to improve extrapulmonary manifestations of CF. Our objectives were to identify reasons for prescribing ETI after LT and evaluate changes in body mass index (BMI), hemoglobin A1c, hemoglobin, and liver enzymes.

methodsThis was an electronic health record-based cohort study, October 2019-September 2020, at 14 CF LT Consortium sites in North America. The study included CF LT recipients prescribed ETI after transplant. Differences in BMI, A1c, and hemoglobin were assessed with paired t-tests.

resultsThere were 94 patients prescribed ETI; indications included sinus disease (68%), GI symptoms (39%), or low BMI (19%). Prescriptions were written by CF physicians (34%), LT physicians (27%), or physicians who practice both CF and LT (39%). Forty patients (42%) stopped ETI at a median of 56 days [IQR 26, 139] after start/prescription date. ETI was not associated with a significant change in BMI (0.2 kg/m

conclusionsETI is rarely prescribed for non-pulmonary indications after LT for CF. Further study is needed to determine the risks and benefits of ETI in the CF lung transplant population given the potential for drug interactions, side effects leading to discontinuation of ETI, and the possible mechanisms for ETI to positively impact long-term post-transplant outcomes.

Indexed as

Cystic FibrosisAminophenolsBenzodioxolesChloride Channel AgonistsCohort StudiesCystic Fibrosis Transmembrane Conductance RegulatorGlycated HemoglobinHumansIndolesLungMutationPyrazolesPyridinesPyrrolidinesQuinolonesTransaminasesAminophenolsBenzodioxolesChloride Channel AgonistsCystic Fibrosis Transmembrane Conductance RegulatorelexacaftorGlycated HemoglobinIndolesivacaftorPyrazolesPyridinesPyrrolidinesQuinolonestezacaftorTransaminasesCystic fibrosis transmembrane conductance regulatorLung transplantationTrikafta

Identifiers

PMID35474016
PMCPMC9509406
OpenAlexW4224982000

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

None linked

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.