Evidence map›Paper›PMID 37699420›Full record

ReviewThe Lancet. Respiratory medicine2023

Understanding and addressing the needs of people with cystic fibrosis in the era of CFTR modulator therapy.

Katherine B Hisert, Susan E Birket, John Paul Clancy, Damian G Downey, John F Engelhardt, Isabelle Fajac, Robert D Gray, Marrah E Lachowicz-Scroggins, Nicole Mayer-Hamblett, Patrick Thibodeau and 3 more

Open access · greenAbstract readReview
In one paragraph

Review in The Lancet. Respiratory medicine, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers.

0numbers the graph read from it
0cells of the map it votes in
34citing papers in PubMed
13.7field-weighted citation impact, top 1% 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

34 citing papers in PubMed, 53 citations in OpenAlex.

  1. Review
  2. Observational
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  16. Evolving nutrition therapy in cystic fibrosis: Adapting to the CFTR modulator era.Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition · 2025
    Review
  17. Article
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4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

13 authors at 10 institutions in 5 countries.

Katherine B HisertDepartment of Medicine, National Jewish Health, Denver, CO, USA. Electronic address: hisertk@njhealth.org.
Susan E BirketDepartment of Medicine, University of Alabama at Birmingham, Birmingham, AL, USA.
John Paul ClancyCystic Fibrosis Foundation, Bethesda, MD, USA.
Damian G DowneyWellcome-Wolfson Institute for Experimental Medicine, School of Medicine, Dentistry and Biomedical Sciences, Queen's University Belfast, Belfast, Northern Ireland.
John F EngelhardtDepartment of Anatomy and Cell Biology, University of Iowa Carver College of Medicine, Iowa City, IA, USA.
Isabelle FajacAssistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France.
Robert D GrayInstitution of Regeneration and Repair, Centre for Inflammation Research, The University of Edinburgh, Edinburgh, UK.
Marrah E Lachowicz-ScrogginsNational Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Nicole Mayer-HamblettDepartment of Pediatrics, Department of Biostatistics, Seattle Children's Research Institute, University of Washington, Seattle, WA, USA.
Patrick ThibodeauCystic Fibrosis Foundation, Bethesda, MD, USA.
Katherine L TuggleCystic Fibrosis Foundation, Bethesda, MD, USA.
Claire E WainwrightUniversity of Queensland, South Brisbane, QLD, Australia.
Kris De BoeckUniversity of Leuven, Leuven, Belgium.
Cystic Fibrosis Foundation · USCentre for Inflammation Research · GBKU Leuven · BENational Institutes of Health · USSeattle Children's Hospital · USUniversité Paris Cité · FRUniversity of Alabama at Birmingham · USUniversity of Colorado Denver · USUniversity of Iowa · USUniversity of Queensland · AU

Funding

Vector Core-Core 2P30DK054759 · NIDDK · UNIVERSITY OF IOWA · PI Alejandro Antonio Pezzulo · 1998 to 2026
$30.5M
Early Pathogenesis of Cystic Fibrosis Related DiabetesRC2DK124207 · NIDDK · UNIVERSITY OF IOWA · PI ENGELHARDT, JOHN F, NORRIS, ANDREW W · 2021 to 2023
$4.5M
Biology of Submucosal Gland Stem Cells in the AirwayR01HL165404 · NHLBI · UNIVERSITY OF IOWA · PI ENGELHARDT, JOHN F · 2022 to 2025
$2.9M
GM-CSF, macrophages, and susceptibility to Mycobacterium abscessus pulmonary infectionR01HL167956 · NHLBI · NATIONAL JEWISH HEALTH · PI Katherine B Hisert · 2023 to 2026
$2.4M
NATIONAL FERRET RESOURCE AND RESEARCH CENTER ON LUNG DISEASE75N92019C00010 · NHLBI · UNIVERSITY OF IOWA · PI BOYLE, JESSICA · 2023 to 2023
$1.1M
Modulating macrophage-mediated Inflammation in cystic fibrosisK08HL136786 · NHLBI · UNIVERSITY OF WASHINGTON · PI HISERT, KATHERINE B · 2018 to 2022
$797k
NHLBI NIH HHS 75N92019C00010NHLBI NIH HHS K08 HL136786NHLBI NIH HHS R01 HL165404NHLBI NIH HHS R01 HL167956NIDDK NIH HHS P30 DK054759NIDDK NIH HHS RC2 DK124207
6 · The paper itself

Abstract

Cystic fibrosis is a multiorgan disease caused by impaired function of the cystic fibrosis transmembrane conductance regulator (CFTR). Since the introduction of the CFTR modulator combination elexacaftor-tezacaftor-ivacaftor (ETI), which acts directly on mutant CFTR to enhance its activity, most people with cystic fibrosis (pwCF) have seen pronounced reductions in symptoms, and studies project marked increases in life expectancy for pwCF who are eligible for ETI. However, modulator therapy has not cured cystic fibrosis and the success of CFTR modulators has resulted in immediate questions about the new state of cystic fibrosis disease and clinical challenges in the care of pwCF. In this Series paper, we summarise key questions about cystic fibrosis disease in the era of modulator therapy, highlighting state-of-the-art research and clinical practices, knowledge gaps, new challenges faced by pwCF and the potential for future health-care challenges, and the pressing need for additional therapies to treat the underlying genetic or molecular causes of cystic fibrosis.

Indexed as

Cystic FibrosisAminophenolsBenzodioxolesChloride Channel AgonistsCystic Fibrosis Transmembrane Conductance RegulatorDelivery of Health CareHumansMutationAminophenolsBenzodioxolesCFTR protein, humanChloride Channel AgonistsCystic Fibrosis Transmembrane Conductance Regulator

Identifiers

PMID37699420
PMCPMC13005650
OpenAlexW4386575375

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.