ArticleAmerican journal of respiratory and critical care medicine2012
Future directions in early cystic fibrosis lung disease research: an NHLBI workshop report.
Article in American journal of respiratory and critical care medicine, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
What it found
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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.
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Who cites it
27 citing papers in PubMed, 69 citations in OpenAlex.
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- V-Type ATPase Mediates Airway Surface Liquid Acidification in Pig Small Airway Epithelial Cells.American journal of respiratory cell and molecular biology · 2021Article
- Quantification of Phenotypic Variability of Lung Disease in Children with Cystic Fibrosis.Genes · 2021Review
- Two Siblings Homozygous for F508del-CFTR Have Varied Disease Phenotypes and Protein Biomarkers.International journal of molecular sciences · 2021Article
- F1099L-CFTR (c.3297C>G) has Impaired Channel Function and Associates with Mild Disease Phenotypes in Two Pediatric Patients.Life (Basel, Switzerland) · 2021Article
- Current state of the art MRI for the longitudinal assessment of cystic fibrosis.Journal of magnetic resonance imaging : JMRI · 2020Review
- Multicentre feasibility of multiple-breath washout in preschool children with cystic fibrosis and other lung diseases.ERJ open research · 2020Article
- Acid exposure disrupts mucus secretion and impairs mucociliary transport in neonatal piglet airways.American journal of physiology. Lung cellular and molecular physiology · 2020Article
- Viral Vectors, Animal Models, and Cellular Targets for Gene Therapy of Cystic Fibrosis Lung Disease.Human gene therapy · 2020Review
- Prospective multicenter randomized patient recruitment and sample collection to enable future measurements of sputum biomarkers of inflammation in an observational study of cystic fibrosis.BMC medical research methodology · 2019Observational
- RNA sequencing data from neutrophils of patients with cystic fibrosis reveals potential for developing biomarkers for pulmonary exacerbations.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2019Article
- AGTR2 absence or antagonism prevents cystic fibrosis pulmonary manifestations.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2019Article
- Personalized medicine in CF: from modulator development to therapy for cystic fibrosis patients with rare CFTR mutations.American journal of physiology. Lung cellular and molecular physiology · 2018Review
- CFTR-NHERF2-LPA₂ Complex in the Airway and Gut Epithelia.International journal of molecular sciences · 2017Review
- AJRCCM: 100-Year Anniversary. Progress along the Pathway of Discovery Leading to Treatment and Cure of Cystic Fibrosis.American journal of respiratory and critical care medicine · 2017Article
- Identification of Bacteria in the Sputum of a Cystic Fibrosis patient; A Comparison of Phenotypic and Molecular Methods.The open microbiology journal · 2017Article
- Early pulmonary disease manifestations in cystic fibrosis mice.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2016Article
Corrections and comments
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Authors and funding
17 authors at 13 institutions in 1 country.
Funding
Abstract
Since the 1989 discovery that mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause cystic fibrosis (CF), there has been substantial progress toward understanding the molecular basis for CF lung disease, leading to the discovery and development of new therapeutic approaches. However, the earliest impact of the loss of CFTR function on airway physiology and structure and its relationship to initial infection and inflammation are poorly understood. Universal newborn screening for CF in the United States represents an unprecedented opportunity for investigating CF clinical manifestations very early in life. Recently developed animal models with pulmonary phenotypic manifestations also provide a window into the early consequences of this genetic disorder. For these reasons, the National Heart, Lung, and Blood Institute (NHLBI) convened a working group of extramural experts, entitled "Future Research Directions in Early CF Lung Disease" on September 21-22, 2010, to identify future research directions of great promise in CF. The priority areas identified included (1) exploring pathogenic mechanisms of early CF lung disease; (2) leveraging newborn screening to elucidate the natural history of early lung disease; (3) developing a spectrum of biomarkers of early lung disease that reflects CF pathophysiology, clinical outcome, and response to treatment; (4) exploring the role of genetics/genomics (e.g., modifier genes, gene-environmental interactions, and epigenetics) in early CF pathogenesis; (5) defining early microbiological events in CF lung disease; and (6) elucidating the initial airway inflammatory, remodeling, and repair mechanisms in CF lung disease.
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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.