ReviewPulmonary therapy2025
Impact of CFTR Modulators on Longitudinal Cystic Fibrosis Survival and Mortality: Review and Secondary Analysis.
Review in Pulmonary therapy, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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.
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.
Who cites it
7 citing papers in PubMed.
- Combining Gene Therapy with Current Modulator Treatments for Cystic Fibrosis: A Promising Area of Research.Pharmaceutics · 2026Review
- Cystic Fibrosis Newborn Screening: Cost Trajectory as a Marker to Improve Health Outcomes.Pediatric pulmonology · 2026Article
- Impact of Highly Effective CFTR Modulator Therapy on Physical Activity, Sleep, and Sinonasal Symptoms in Preschool Children with Cystic Fibrosis: A Prospective Single-Center Pilot Study.Advances in respiratory medicine · 2026Observational
- Pathophysiology of cystic fibrosis-related liver disease.Current opinion in gastroenterology · 2026Review
- Review
- Redefining Cystic Fibrosis-Related Diabetes Management With Tirzepatide: A Case Report.Cureus · 2025Article
- Cystic Fibrosis and CFTR Modulators: The Impact on Bone Density, Muscle Mass and Strength in Children and Young Adolescents.Children (Basel, Switzerland) · 2025Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors.
Funding
Abstract
introductionCystic fibrosis (CF) transmembrane conductance regulator modulators (CFTRm) have transformed CF care, shifting treatment from only managing symptoms to also addressing the underlying defects that cause CF. CFTRm first entered clinical practice in 2012 and was followed by additional CFTRm combinations-including the approval of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) in 2019-which treats most CF genotypes.
methodsWe identified peer-reviewed literature for a narrative review (January 1990 to January 2025) describing longitudinal trends in CF survival and age of death and assessing the influence of CFTRm, particularly ELX/TEZ/IVA. To supplement the existing literature, a secondary analysis of historical, longitudinal trends in the United States CF Foundation Patient Registry (U.S. CFFPR, 1990-2023) was conducted using recent available data.
resultsQuantitative data from published studies show that the median age of survival and death increased over time but with varying magnitudes across regions. Most cohort and registry-based studies were conducted in settings where CFTRm were not yet widely available, limiting the evaluation of CFTRm effects on survival trends over time. In the secondary U.S. CFFPR analysis, the median survival age increased from 29.0 years in 1990 to 38.6 years in 2012 prior to the introduction of CFTRm and to 68.0 years in 2023, demonstrating substantial improvement following the introduction of CFTRm. Linear regression analyses showed gains in median survival age increased from 0.48 years per year prior to CFTRm to 4.79 years per year after approval of ELX/TEZ/IVA in 2019.
conclusionsStudy results provide initial evidence of the impact of CFTRm to meaningfully improve survival. Longer-term follow-up data across geographies will provide a deeper understanding of the full impact of CFTRm on predicted CF survival and mortality.
Indexed as
Identifiers
What OpenQuestion holds
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.