Evidence map›Paper›PMID 42729189›Full record

ArticleFrontiers in medicine2026

Clinical benefit without sweat chloride response after ETI therapy in an adult with cystic fibrosis bearing the L467F;F508del complex

Marcela Kreslova, Daan Caudri, Isabelle Sermet-Gaudelus, Aurelie Hatton, Punitkumar Makani, Vaclav Koucky, Malgorzata Libik, Hana Hrdinova, Libor Fila, Nela Stastna and 5 more

Abstract readCase Reports
PubMed Full text at PMC Publisher
In one paragraph

Article in Frontiers in medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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

0 citing papers in PubMed.

No citing paper in PubMed yet.

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

15 authors.

Marcela KreslovaDepartment of Paediatrics, University Hospital, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czechia.
Daan CaudriDepartment of Paediatrics, Division of Respiratory Medicine and Allergy, Erasmus MC-Sophia Children's Hospital, University Medical Centre Rotterdam, Rotterdam, Netherlands.
Isabelle Sermet-GaudelusInstitut Necker Enfants Malades, INSERM, Université Paris Cité, Paris, France.
Aurelie HattonInstitut Necker Enfants Malades, INSERM, Université Paris Cité, Paris, France.
Punitkumar MakaniDepartment of Paediatrics, Division of Respiratory Medicine and Allergy, Erasmus MC-Sophia Children's Hospital, University Medical Centre Rotterdam, Rotterdam, Netherlands.
Vaclav KouckyDepartment of Paediatrics, Second Faculty of Medicine, Charles University, Motol and Homolka University Hospital, Prague, Czechia.
Malgorzata LibikDepartment of Biology and Medical Genetics, Second Faculty of Medicine, Charles University, Motol and Homolka University Hospital, Prague, Czechia.
Hana HrdinovaDepartment of Imaging Methods, Second Faculty of Medicine, Charles University, Motol and Homolka University Hospital, Prague, Czechia.
Libor FilaDepartment of Pneumology, Second Faculty of Medicine, Charles University, Motol and Homolka University Hospital, Prague, Czechia.
Nela StastnaDepartment of Pneumology, Second Faculty of Medicine, Charles University, Motol and Homolka University Hospital, Prague, Czechia.
Eva FurstovaDepartment of Paediatrics, First Faculty of Medicine, Charles University, Thomayer University Hospital, Prague, Czechia.
Radka BittenglovaDepartment of Pneumology, University Hospital, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czechia.
Jan BaxaDepartment of Imaging Methods, NH Hospital, AKESA Holding, Horovice, Czechia.
Josef SykoraDepartment of Paediatrics, University Hospital, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czechia.
Milan MacekDepartment of Biology and Medical Genetics, Second Faculty of Medicine, Charles University, Motol and Homolka University Hospital, Prague, Czechia.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: Elexacaftor/tezacaftor/ivacaftor (ETI) is the standard of care for most people with cystic fibrosis (CF) who carry at least one F508del allele. However, predicting responses to CFTR modulators in rare or complex Case presentation: We describe an 8-year longitudinal pre- and post-treatment assessment of an adult woman with CF, adherent to therapy, with the L467F;F508del complex allele compounded with the non-responsive c.489 + 1G > T (621 + 1G > T) variant in Conclusion: Persistent CFTR biomarker negativity may not fully exclude clinically meaningful ETI-associated benefit in rare or complex

Indexed as

AI-assisted CTcase reportcomplex CFTR allelecystic fibrosisETI (elexacaftor/tezacaftor/ivacaftor)

Identifiers

PMID42729189
PMCPMC13561797

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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.