Evidence map›Paper›PMID 40024881›Full record

ArticleInternational forum of allergy & rhinology2025

Elexacaftor-Tezacaftor-Ivacaftor Improves Sinonasal Outcomes in Young Children With Cystic Fibrosis.

Amanda L Stapleton, Adam Kimple, Jennifer L Goralski, Daniel M Beswick, Anshika Gupta, Douglas A Li, Barton F Branstetter, S Mehdi Nouraie, Amber D Shaffer, Brent Senior and 1 more

Abstract readMulticenter Study
In one paragraph

Article in International forum of allergy & rhinology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
9citing papers in PubMed, 1 pooled it
–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

9 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. Article
  3. Observational
  4. Article
  5. Observational
  6. Article
  7. Article
  8. Review
  9. 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

11 authors.

Amanda L StapletonDepartment of Otolaryngology - Head & Neck Surgery, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
Adam KimpleDepartment of Otolaryngology - Head & Neck Surgery, University of North Carolina, Chapel Hill, North Carolina, USA.
Jennifer L GoralskiDivision of Pediatric Pulmonology, University of North Carolina, Chapel Hill, North Carolina, USA.
Daniel M BeswickDepartment of Head and Neck Surgery, University of California Los Angeles, Los Angeles, California, USA.ORCID 0000-0001-8612-5442
Anshika GuptaDepartment of Microbiology and Molecular Genetics, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
Douglas A LiDivision of Pediatric Pulmonology, University of California Los Angeles, Los Angeles, California, USA.
Barton F BranstetterDepartment of Otolaryngology - Head & Neck Surgery, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
S Mehdi NouraieDivision of Pulmonary, Allergy, Critical Care, and Sleep Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
Amber D ShafferDepartment of Otolaryngology - Head & Neck Surgery, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.ORCID 0000-0001-5783-9198
Brent SeniorDepartment of Otolaryngology - Head & Neck Surgery, University of North Carolina, Chapel Hill, North Carolina, USA.ORCID 0000-0002-1503-3150
Anna C ZemkeDivision of Pulmonary, Allergy, Critical Care, and Sleep Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.ORCID 0000-0002-7722-6708

Funding

Cystic Fibrosis Foundation ZEMKE19A0University of Pittsburgh UL1-TR-001857
6 · The paper itself

Abstract

backgroundSevere chronic rhinosinusitis (CRS) is a near universal manifestation of cystic fibrosis. Elexacaftor/tezacaftor/ivacaftor (ETI) is an oral, small molecule, highly effective Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) corrector-potentiator drug. In people with cystic fibrosis age > 12 years, ETI improves sinonasal symptoms, endoscopy findings, polyp size, and radiologic findings. This study evaluates changes in CRS in children ages 6-12 years newly started on ETI.

methodsThis was a prospective, three center, pre-post study of 11 children age 6-11 years newly started on ETI. Study endpoints included the SN-5 sinonasal health survey, Sniffin' Kids olfaction test, a sinus computerized tomography (CT) scan, and nasal endoscopy with mucus sampling for full-length 16S rRNA sequencing microbiome analysis. Study visits were conducted before ETI and at a median of 9 months after treatment initiation.

resultsETI lead to improvement in symptoms, endoscopy scores and radiologic findings of CRS. Olfaction was below normal at baseline and did not improve. The sinonasal microbiome was dominated by typically commensal organisms before and after treatment for most participants. Additionally, Staphylococcus aureus was found in five participants at baseline and six participants on treatment.

conclusionsETI improves sinonasal symptoms and endoscopy findings in children 6-11 years of age. Olfaction did not improve with ETI treatment in this age group, suggesting that olfactory dysfunction associated with CF is established early in life. This younger cohort of pediatric patients presented with abundant Staphylococcus aureus and only very rare Pseudomonas aeruginosa at baseline or after treatment.

Indexed as

AminophenolsBenzodioxolesCystic FibrosisIndolesPyrazolesPyridinesQuinolonesRhinitisSinusitisChildChloride Channel AgonistsChronic DiseaseDrug CombinationsFemaleHumansMaleAminophenolsBenzodioxolesChloride Channel AgonistsDrug Combinationselexacaftor, ivacaftor, tezacaftor drug combinationIndolesPyrazolesPyridinesQuinolinesQuinolonesendoscopyolfactionPseudomonas aeruginosarhinosinusitisStaphylococcus aureus

Identifiers

PMID40024881
PMCPMC12227111

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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.