Evidence map›Paper›PMID 38719441›Full record

Observational studyThorax2024

Impact of age at ivacaftor initiation on pulmonary outcomes among people with cystic fibrosis.

Christian A Merlo, Lisa J McGarry, Teja Thorat, Catherine Nguyen, Maral DerSarkissian, Aruna Muthukumar, Joe Healy, M Alan Brookhart, Jaime L Rubin

Abstract readObservational Study
In one paragraph

Observational study in Thorax, 2024. 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. Trial
  2. Review
  3. Article
  4. Cystic Fibrosis Year in Review 2024.Pediatric pulmonology · 2025
    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

9 authors.

Christian A MerloJohns Hopkins School of Medicine, Baltimore, Maryland, USA cmerlo@jhmi.edu.ORCID 0000-0002-9283-3928
Lisa J McGarryVertex Pharmaceuticals Incorporated, Boston, Massachusetts, USA.
Teja ThoratVertex Pharmaceuticals Incorporated, Boston, Massachusetts, USA.
Catherine NguyenAnalysis Group Inc, Los Angeles, California, USA.
Maral DerSarkissianAnalysis Group Inc, Los Angeles, California, USA.
Aruna MuthukumarAnalysis Group Inc, Menlo Park, California, USA.
Joe HealyVertex Pharmaceuticals Incorporated, Boston, Massachusetts, USA.
M Alan BrookhartDuke University, Durham, North Carolina, USA.
Jaime L RubinVertex Pharmaceuticals Incorporated, Boston, Massachusetts, USA.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundIvacaftor (IVA) improves lung function and other extrapulmonary outcomes in people with cystic fibrosis (CF). However, the effect of initiating IVA at earlier versus later ages has not been studied.

methodsWe conducted an observational cohort study of people in the US CF Foundation Patient Registry aged ≥6 years with ≥1 CF transmembrane conductance regulator-gating mutation to compare the effects of initiating IVA at earlier ages on per cent predicted forced expiratory volume in 1 s (ppFEV

findingsBaseline characteristics were well balanced between younger and older IVA initiator groups after SMR weighting. In the outcome assessment period, younger IVA initiators had significantly higher mean ppFEV

interpretationStudy findings showed the importance of early IVA initiation in people with CF.

Indexed as

AminophenolsCystic FibrosisQuinolonesAdolescentAdultAge FactorsChildChloride Channel AgonistsCystic Fibrosis Transmembrane Conductance RegulatorDisease ProgressionFemaleForced Expiratory VolumeHumansMaleMutationRegistriesAminophenolsChloride Channel AgonistsCystic Fibrosis Transmembrane Conductance RegulatorivacaftorQuinolonesCystic Fibrosis

Identifiers

PMID38719441
PMCPMC11503177

What OpenQuestion holds

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LicenceCC BY-NC
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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.