Evidence map›Paper›PMID 38448281›Full record

ArticleJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2024

Alterations in the fecal microbiota in patients with advanced cystic fibrosis liver disease after 6 months of elexacaftor/tezacaftor/ivacaftor.

Jennifer T Duong, Christopher E Pope, Hillary S Hayden, Carson Miller, Stephen J Salipante, Steven M Rowe, George M Solomon, David Nichols, Lucas R Hoffman, Michael R Narkewicz and 1 more

Registry-linked trialOpen access · bronzeAbstract readEvaluation Study
PubMed Full text at PMC Publisher
In one paragraph

Article in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT04038047 (A Prospective Study to Evaluate Biological and Clinical Effects of Significantly Corrected CFTR Function), which is not on this map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
4.6field-weighted citation impact, top 5% 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.

NCT04038047 completednot on this map

A Prospective Study to Evaluate Biological and Clinical Effects of Significantly Corrected CFTR Function (The PROMISE Study)

TypeobservationalSponsorNicole HamblettRan2019 to 2024Enrolled490ConditionsCystic Fibrosis
3 · Its place in the literature

Who cites it

4 citing papers in PubMed, 11 citations in OpenAlex.

  1. Pathophysiology of cystic fibrosis-related liver disease.Current opinion in gastroenterology · 2026
    Review
  2. Observational
  3. Review
  4. 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

11 authors at 5 institutions in 1 country.

Jennifer T DuongDepartment of Pediatrics, Division of Pediatric Gastroenterology, Hepatology, and Nutrition, University of California San Francisco School of Medicine, San Francisco, CA, USA. Electronic address: Jennifer.duong@ucsf.edu.
Christopher E PopeDepartment of Microbiology, University of Washington School of Medicine, Seattle, WA, USA.
Hillary S HaydenDepartment of Microbiology, University of Washington School of Medicine, Seattle, WA, USA.
Carson MillerDepartment of Microbiology, University of Washington School of Medicine, Seattle, WA, USA.
Stephen J SalipanteDepartment of Laboratory Medicine and Pathology, University of Washington School of Medicine, Seattle, WA, USA.
Steven M RoweDepartment of Medicine and the Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA.
George M SolomonDepartment of Medicine and the Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA.
David NicholsCystic Fibrosis Foundation Therapeutics Development Network Coordinating Center, Seattle Children's Research Institute, Seattle, WA, USA and Department of Pediatrics, University of Washington School of Medicine, Seattle, WA, USA.
Lucas R HoffmanDepartment of Microbiology and Department of Pediatrics, Division of Pulmonary and Sleep Medicine, University of Washington School of Medicine, Seattle, WA, USA.
Michael R NarkewiczDigestive Health Institute, Children's Hospital Colorado and Section of Pediatric Gastroenterology, Hepatology and Nutrition, University of Colorado SOM, Aurora, CO, USA.
Nicole GreenDepartment of Pediatrics, Division of Pediatric Gastroenterology, Hepatology, and Nutrition, University of Washington School of Medicine, Seattle, WA, USA.
University of Washington · USUniversity of Alabama at Birmingham · USChildren's Hospital Colorado · USCystic Fibrosis Foundation · USUniversity of California, San Francisco · US

Funding

UAB CF Research and Translation Core CenterP30DK072482 · NIDDK · UNIVERSITY OF ALABAMA AT BIRMINGHAM · PI Brian R. Davis · 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
Patient-oriented microbiome and advanced culture approaches to identifying the microbial determinants of chronic pediatric diseaseK24HL141669 · NHLBI · UNIVERSITY OF WASHINGTON · PI Lucas R Hoffman · 2018 to 2026
$929k
NHLBI NIH HHS K24 HL141669NIDDK NIH HHS P30 DK072482NIDDK NIH HHS P30 DK089507
6 · The paper itself

Abstract

backgroundCystic fibrosis associated liver disease (CFLD) carries a significant disease burden with no effective preventive therapies. According to the gut-liver axis hypothesis for CFLD pathogenesis, dysbiosis and increased intestinal inflammation and permeability permit pathogenic bacterial translocation into the portal circulation, leading to hepatic inflammation and fibrosis. Evaluating the effect of CFTR (cystic fibrosis transmembrane conductance regulator) modulation with elexacaftor/tezacaftor/ivacaftor (ETI) may help determine the role of CFTR in CFLD and increase understanding of CFLD pathogenesis, which is critical for developing therapies. We aimed to characterize the fecal microbiota in participants with CF with and without advanced CFLD (aCFLD) before and after ETI.

methodsThis is an ancillary analysis of stool samples from participants ages ≥12 y/o enrolled in PROMISE (NCT04038047). Included participants had aCFLD (cirrhosis with or without portal hypertension, or non-cirrhotic portal hypertension) or CF without liver disease (CFnoLD). Fecal microbiota were defined by shotgun metagenomic sequencing at baseline and 1 and 6 months post-ETI.

resultsWe analyzed 93 samples from 34 participants (11 aCFLD and 23 CFnoLD). Compared to CFnoLD, aCFLD had significantly higher baseline relative abundances of potential pathogens Streptococcus salivarius and Veillonella parvula. Four of 11 aCFLD participants had an initially abnormal fecal calprotectin that normalized 6 months post-ETI, correlating with a significant decrease in S. salivarius and a trend towards decreasing V. parvula.

conclusionsThese results support an association between dysbiosis and intestinal inflammation in CFLD with improvements in both post-ETI, lending further support to the gut-liver axis in aCFLD.

Indexed as

AminophenolsBenzodioxolesCystic FibrosisFecesGastrointestinal MicrobiomeIndolesQuinolonesAdolescentAdultChloride Channel AgonistsDrug CombinationsDysbiosisFemaleHumansLiver DiseasesMaleAminophenolsBenzodioxolesChloride Channel AgonistsDrug CombinationselexacaftorIndolesivacaftorPyrazolesPyridinesPyrrolesPyrrolidinesQuinolonestezacaftorCystic fibrosis liver diseaseDysbiosisElexacaftor/tezacaftor/ivacaftorFecal microbiomeGut-liver axis

Identifiers

PMID38448281
PMCPMC11182717
OpenAlexW4392501783

What OpenQuestion holds

Textmetadata
LicenceTDM
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.