ReviewAnnals of the American Thoracic Society2018
Cystic Fibrosis: Translating Molecular Mechanisms into Effective Therapies.
Review in Annals of the American Thoracic Society, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 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
16 citing papers in PubMed, 23 citations in OpenAlex.
- Remodeling the pathological airway: advanced nanotechnology for diagnostics and therapeutics in cystic fibrosis.Journal of nanobiotechnology · 2026Review
- Repurposing ivacaftor to attenuate LPS-induced acute lung injury: evidence from a non-cystic fibrosis model.Frontiers in pharmacology · 2026Article
- Real-life impact of genotype and severity of lung disease on efficacy of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis.Pulmonary pharmacology & therapeutics · 2025Observational
- The effect of modulators on lung function following inpatient treatment for CF exacerbations.Frontiers in pediatrics · 2025Article
- Recommended Tool Compounds for Modifying the Cystic Fibrosis Transmembrane Conductance Regulator Channel Variants.ACS pharmacology & translational science · 2024Review
- Resolution of long standing hypergammaglobulinemic purpura of Waldenström after initiating treatment for cystic fibrosis with Elexacaftor-Tezacaftor-Ivacaftor.JAAD case reports · 2024Article
- Dry Powder Inhalation for Lung Delivery in Cystic Fibrosis.Pharmaceutics · 2023Review
- N-acetylcysteine (NAC) and Its Role in Clinical Practice Management of Cystic Fibrosis (CF): A Review.Pharmaceuticals (Basel, Switzerland) · 2022Review
- New drugs in cystic fibrosis: what has changed in the last decade?Therapeutic advances in chronic disease · 2022Review
- Airway Inflammation and Host Responses in the Era of CFTR Modulators.International journal of molecular sciences · 2020Review
- Overcoming Immunological Challenges to Helper-Dependent Adenoviral Vector-Mediated Long-TermGenes · 2020Article
- TMEM16A: An Alternative Approach to Restoring Airway Anion Secretion in Cystic Fibrosis?International journal of molecular sciences · 2020Review
- The Extrapulmonary Effects of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Cystic Fibrosis.Annals of the American Thoracic Society · 2020Review
- Evaluation of 1,2,3-Triazoles as Amide Bioisosteres In Cystic Fibrosis Transmembrane Conductance Regulator Modulators VX-770 and VX-809.Chemistry (Weinheim an der Bergstrasse, Germany) · 2019Article
- Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota.Cellular and molecular gastroenterology and hepatology · 2019Review
- Consuming Genistein Improves Survival Rates in the Absence of Laxative in ΔF508-CF Female Mice.Nutrients · 2018Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 2 institutions in 1 country.
Funding
Abstract
Cystic fibrosis is a genetic disease that affects approximately 75,000 individuals around the world. Long regarded as a lethal and life-limiting disease, with the most severe manifestations expressed in the progressive decline of lung function, treatment advances focusing on airway clearance and management of chronic lung infection have resulted in improved outcomes for individuals with cystic fibrosis. These advances have been realized in conjunction with an improved understanding of the genetic basis of this disease, dating back to the discovery of the cystic fibrosis gene in 1989. The identification of the cystic fibrosis gene and the advancement of our understanding of the resultant cystic fibrosis transmembrane conductance regulator protein have led to the development of a new class of cystic fibrosis therapies designed to directly impact the function of this protein. These therapeutic developments have progressed, targeting the various mutations that can cause cystic fibrosis. These new medications, known as cystic fibrosis transmembrane conductance regulator modulators, have changed the landscape of cystic fibrosis care and cystic fibrosis research. Their demonstrated effect in patients with specific cystic fibrosis mutations has ignited the hope that such therapies will soon be available to more individuals with this disease, moving the cystic fibrosis community significantly closer to the ultimate goal of curing this disease.
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.