Evidence map›Paper›PMID 33619012›Full record

ReviewJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2021

PROMISE: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy.

Dave P Nichols, Scott H Donaldson, Carla A Frederick, Steven D Freedman, Daniel Gelfond, Lucas R Hoffman, Andrea Kelly, Michael R Narkewicz, Jessica E Pittman, Felix Ratjen and 10 more

Registry-linked trialAbstract readReview
In one paragraph

Review in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT06322446 (Effects of a Strength Exercise Program on Muscular Health in People With CF Treated With Modulators of the Cystic Fibrosis Transmembrane Conductance Regulator Receptor), which is not on this map. Cited by 51 papers.

0numbers the graph read from it
0cells of the map it votes in
51citing 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.

NCT06322446 naactive not recruitingnot on this mapstarted 2024, after this paper: background citation

Effects of a Strength Exercise Program on Muscular Health in People With CF Treated With Modulators of the Cystic Fibrosis Transmembrane Conductance Regulator Receptor (CFTR)

TypeinterventionalSponsorUniversidad Politecnica de MadridRan2024 to 2026Enrolled48ConditionsCystic FibrosisArmsExercise
3 · Its place in the literature

Who cites it

51 citing papers in PubMed.

  1. Article
  2. Review
  3. Observational
  4. Article
  5. Article
  6. Review
  7. Observational
  8. Article
  9. Observational
  10. Observational
  11. Cystic fibrosis-related bone disease: an update on screening, diagnosis, and treatment.Therapeutic advances in endocrinology and metabolism · 2025
    Review
  12. Article
  13. Article
  14. 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
  15. Article
  16. Article
  17. Article
  18. Article
  19. Effect of elexacaftor/tezacaftor/ivacaftor on mucus and mucociliary clearance in cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2024
    Observational
  20. Article
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

20 authors.

Dave P NicholsSeattle Children's Research Institute, Department of Pediatrics, University of Washington, USA. Electronic address: david.nichols@seattlechildrens.org.
Scott H DonaldsonDepartment of Medicine, Univ. North Carolina at Chapel Hill, USA.
Carla A FrederickJacobs School of Medicine and Biomedical Sciences of the University of Buffalo, USA.
Steven D FreedmanBeth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA.
Daniel GelfondWestern New York Pediatric Gastroenterology, USA.
Lucas R HoffmanSeattle Children's Research Institute, Department of Pediatrics and Microbiology, University of Washington, USA.
Andrea KellyDepartment of Endocrinology, University of Pennsylvania, USA.
Michael R NarkewiczDigestive Health Institute, Children's Hospital Colorado and University of Colorado School of Medicine, Aurora, CO, USA.
Jessica E PittmanDepartment of Pediatrics, Washington University School of Medicine, USA.
Felix RatjenDepartment of Pediatrics, University of Toronto, USA.
Scott D SagelSeattle Children's Research Institute, Department of Pediatrics and Epidemiology, University of Washington, USA.
Margaret RosenfeldDepartment of Pediatrics, Children's Hospital Colorado and University of Colorado School of Medicine, Aurora, CO, USA.
Sarah Jane SchwarzenbergUniversity of Minnesota Masonic Children's Hospital, Minneapolis, MN, USA.
Pradeep K SinghDepartment of Microbiology, University of Washington, USA.
George M SolomonGregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA.
Michael S StalveyGregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA.
Shannon KirbyTherapeutics Development Network Coordinating Center, Seattle Children's Hospital, USA.
Jill M VanDalfsenTherapeutics Development Network Coordinating Center, Seattle Children's Hospital, USA.
John P ClancyCystic Fibrosis Foundation, USA.
Steven M RoweGregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA.

Funding

UAB CF Research and Translation Core CenterP30DK072482 · NIDDK · UNIVERSITY OF ALABAMA AT BIRMINGHAM · PI AMIT GAGGAR · 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
NIDDK NIH HHS P30 DK072482NIDDK NIH HHS P30 DK089507
6 · The paper itself

Abstract

Highly effective CFTR modulator drug therapy is increasingly available to those with cystic fibrosis. Multiple observational research studies are now being conducted to better understand the impacts of this important therapeutic milestone on long-term outcomes, patient care needs, and future research priorities. PROMISE is a large, multi-disciplinary academic study focused on the broad impacts of starting elexacaftor/tezacaftor/ivacaftor in the US population age 6 years and older. The many areas of investigation and rationale for each are discussed by organ systems, along with recognition of remaining important questions that will not be addressed by this study alone. Knowledge gained through this and multiple complementary studies around the world will help to understand important health outcomes, clinical care priorities, and research needs for a large majority of people treated with these or similarly effective medications targeting the primary cellular impairment in cystic fibrosis.

Indexed as

Chloride Channel AgonistsCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorDrug CombinationsHumansObservational Studies as TopicChloride Channel AgonistsCystic Fibrosis Transmembrane Conductance RegulatorDrug CombinationsCftrClinical trialCystic fibrosisDrugElexacaftorIvacaftorModulatorPromiseTezacaftor

Identifiers

PMID33619012
PMCPMC8686210

What OpenQuestion holds

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Read underepoch 390

Registered trials

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.