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1,320 results for “cystic fibrosis”
FEED-Cystic Fibrosis (FEED-CF)
ClinicalTrials.gov study NCT05766774. IPD Sharing: YES. Countries: 1. Publications: 1.
A Study of the Comparable Efficacy and Safety of Pulmozyme (Dornase Alfa) Delivered by the eRapid Nebulizer System in Patients With Cystic Fibrosis
ClinicalTrials.gov study NCT01712334. IPD Sharing: Not stated. Countries: 1. Publications: 2.
Effect of Azithromycin on Lung Function in 6-18 Year-olds With Cystic Fibrosis (CF) Not Infected With P. Aeruginosa
ClinicalTrials.gov study NCT00431964. IPD Sharing: Not stated. Countries: 2. Publications: 2.
Data from: Life in the cystic fibrosis upper respiratory tract influences competitive ability of the opportunistic pathogen Pseudomonas aeruginosa
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Safety and tolerability of a low glycemic load dietary intervention in adults with cystic fibrosis: A pilot study
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Acquired dysfunction of CFTR underlies cystic fibrosis-like disease of the canine gallbladder
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Cystic fibrosis autoantibody signatures associate with Staphylococcus aureus lung infection or cystic fibrosis-related diabetes
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Heterogenous susceptibility to R-pyocins in populations of Pseudomonas aeruginosa sourced from cystic fibrosis lungs
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Personalized inhaled bacteriophage therapy for treatment of multidrug-resistant Pseudomonas aeruginosa in cystic fibrosis
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Cystic fibrosis alters the structure of the olfactory epithelium and the expression of olfactory receptors affecting odor perception
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High prevalence of lipopolysaccharide mutants and R2-Pyocin susceptible variants in Pseudomonas aeruginosa populations sourced from cystic fibrosis lung infections
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Human nasal epithelial organoids for therapeutic development in cystic fibrosis
<p>Table S1: Individual subject demographics and available clinical data for selected participants contributing functional data. Figure S1: Example measurement of µOCT ciliary beat frequencies, Figure S2: Organoid CFTR (green) co-localization with apical ZO-1 (red). (A) To illustrate the location of the ZO-1 staining within the organoid, an alpha-blended 3-D volume reconstruction was created with the fluorescence intensity increased to reveal the organoid’s shape and orient the reader to the location of the 2D co-localization image below. (B) Magnified view of the yellow frame in (A) to better visualize the CFTR and ZO-1 overlay, Video S1: Brightfield video of luminal movement of mucus, Video S2: µOCT video of luminal movement of mucus, Video S3: Brightfield video of luminal cilia beating, Video S4: µOCT video of cilia beating, Video S5: FIS assay of non-CF organoids, Video S6: FIS assay of F508del/P67L organoids. Video S7: FIS assay of F508del/F508del organoids.</p>
Neutrophil extracellular traps are present in the airways of ENaC-overexpressing mice with cystic fibrosis-like lung disease
<p>Background: Neutrophils are key components of the exacerbated inflammation and tissue damage in cystic fibrosis (CF) airways. Neutrophil extracellular traps (NETs) trap and kill extracellular pathogens. While NETs are abundant in the airways of CF patients and have been hypothesized to contribute to lung damage in CF, the in vivo role of NETs remains controversial, partially due to lack of appropriate animal models. The goal of this study was to detect NETs and to further characterize neutrophilmediated inflammation in the airways of mice overexpressing the epithelial sodium channel (βENaC-Tg mice on C57BL/6 background) in their lung with CF-like airway disease, in the absence of any apparent bacterial infections.</p> <p>Methods: Histology scoring of lung tissues, flow cytometry, multiplex ELISA, immunohistochemistry and immunofluorescence were used to characterize NETs and the airway environment in uninfected, βENaC-Tg mice at 6 and 8 weeks of age, the most chronic time points so far studied in this model.</p> <p>Results: Excessive neutrophilic infiltration characterized the lungs of uninfected, βENaC-Tg mice at 6 and 8 weeks of age. The bronchoalveolar lavage fluid (BALF) of βENaC-Tg mice contains increased levels of CF-associated cytokines and chemokines: KC, MIP-1α/β, MCP-1, G-CSF, IL-5, and IL-6. The BALF of βENaC-Tg mice contain MPO-DNA complexes, indicative of the presence of NETs. Immunofluorescence and flow cytometry of BALF neutrophils and lung tissues demonstrated increased histone citrullination, a NET-specific marker, in βENaC-Tg mice.</p> <p>Conclusions: NETs are detected in the airways of βENaC-Tg mice, in the absence of bacterial infections. These data demonstrate the usefulness of the βENaC-Tg mouse to serve as a model for studying the role of NETs in chronic CF airway inflammation.</p> <p>Keywords Cystic fibrosis; neutrophil extracellular traps; NET; ENaC, neutrophil.</p>
Replication Data for: OPTICAL COHERENCE TOMOGRAPHY IDENTIFIES LOWER LABIAL SALIVARY GLAND SURFACE DENSITY IN CYSTIC FIBROSIS
<p>Swept-source optical coherence tomography images of the mucosa of the lower lip acquired in 18 cystic fibrosis patients (CF.zip, includes an XLS file with additional data) and 18 healthy volunteers (HS.zip). Within the volumetric datasets consisting of sets of images the labial salivary glands can be identified.</p> <p>Authors: Nowak JK, Grulkowski I, Karnowski K, Wojtkowski M, Walkowiak J.<br /> When using the data, please always refer to the original paper (currently under review in PLOS ONE; title as specified above).</p> <p>The methodology used to obtain the volumetric datasets is described in:<br /> Grulkowski I, Nowak JK, Karnowski K, Zebryk P, Puszczewicz M, Walkowiak J, Wojtkowski M. Quantitative assessment of oral mucosa and labial minor salivary glands in patients with Sjögren’s syndrome using swept source OCT. Biomedical Optics Express, Vol. 5, Issue 1, pp. 259-274 (2014). DOI: http://dx.doi.org/10.1364/BOE.5.000259</p>
Cystic Fibrosis Diagnosis in Newborns with Machine Learning]{Newborn Cystic Fibrosis Diagnosis Made Accurate and Efficient with Machine Learning to Reduce False Positives in IRT-Trypsinogen Immunoreactive Screening Program
<p>Datasets to training models in this article and generator code.</p>
Continuous glucose monitoring in cystic fibrosis: glycemic relationships, clinical correlations, and implications for CFRD diagnosis
<p>Context: The clinical utility and implications of continuous glucose monitoring (CGM) in cystic fibrosis (CF) are unclear.</p> <p>Objective: We examined the correlation between CGM measures and clinical outcomes in adults with CF, investigated the relationship between hemoglobin A1c (HbA1c) and CGM-derived average glucose (AG), and explored CGM measures that distinguish cystic fibrosis–related diabetes (CFRD) from normal and abnormal glucose tolerance.</p> <p>Methods: This prospective observational study included 77 adults with CF who had CGM and HbA1c measured at 2 to 3 time points 3 months apart.</p> <p>Results: Thirty-one of the 77 participants met American Diabetes Association–recommended diagnostic criteria for CFRD by oral glucose tolerance testing and/or HbA1c. In all participants, CGM measures of hyperglycemia and glycemic variability correlated with nutritional status and pulmonary function. HbA1c was correlated with AG (R2 = 0.71, P < 0.001), with no significant difference between this regression line and that previously established in type 1 and type 2 diabetes and healthy volunteers. Cutoffs of 17.5% time > 140 mg/dL and 3.4% time > 180 mg/dL had sensitivities of 87% and 90%, respectively, and specificities of 95%, for identifying CFRD. Area under the curve and percent of participants correctly classified with CFRD were higher for AG, SD, % time > 140, > 180, and > 250 mg/dL than for HbA1c.</p> <p>Conclusion: CGM measures of hyperglycemia and glycemic variability are superior to HbA1c in distinguishing those with and without CFRD. CGM-derived AG is strongly correlated with HbA1c in adults with CF, with a similar relationship to other diabetes populations. Future studies are needed to investigate CGM as a diagnostic and screening tool for CFRD.</p>
Translation, cross-cultural adaptation, and evaluation of psychometric properties of cystic fibrosis stigma scale
<pre>Search database</pre>
Stenotrophomonas isolates from patients with cystic fibrosis and other chronic respiratory infections
<p><strong>Background:</strong> The potential pathogenic role of <em>Stenotrophomonas maltophilia</em> in lung disease and in particular in cystic fibrosis is unclear. To develop further understanding of the biology of this taxa, the taxonomic position, antibiotic resistance and virulence factors of <em>S. maltophilia</em> isolates from patients with chronic lung disease were studied.</p> <p><strong>Results:</strong> A total of 111 isolates recovered between 2003 and 2016 from respiratory samples from patients in five different countries were included. Based on a cut-off of 95%, analysis of average nucleotide identity by BLAST (ANIb) showed that the 111 isolates identified as <em>S. maltophilia</em> by Matrix-assisted laser desorption/ionization time of flight mass spectrometry (MALDI-TOF/MS) belonged to <em>S. maltophilia</em> (n=65), <em>S. pavanii </em>(n=6) and 13 putative novel species (n=40), which each included 1-5 isolates; these groupings coincided with the results of the 16S rDNA analysis, and the L1 and L2 ß-lactamase Neighbor-Joining phylogeny. Chromosomally encoded aminoglycoside resistance was identified in all <em>S. maltophilia</em> and <em>S. pavani</em> isolates, while acquired antibiotic resistance genes were present in only a few isolates. Nevertheless, phenotypic resistance levels against commonly used antibiotics, determined by standard broth microbroth dilution, were high. Although putative virulence genes were present in all isolates, the percentage of positive isolates varied. The Xps II secretion system responsible for the secretion of the StmPr1-3 proteases was mainly limited to isolates identified as <em>S. maltophilia</em> based on ANIb, but no correlation with phenotypic expression of protease activity was found. The RPF two-component quorum sensing system involved in virulence and antibiotic resistance expression has two main variants with one variant lacking 190 amino acids in the sensing region.</p> <p><strong>Conclusions:</strong> The putative novel <em>Stenotrophomonas</em> species recovered from patient samples and identified by MALDI-TOF/MS as <em>S. maltophilia</em>, differed from <em>S. maltophilia</em> in resistance and virulence genes, and therefore possibly in pathogenicity. Revision of the <em>Stenotrophomonas</em> taxonomy is needed in order to reliably identify strains within the genus and elucidate the role of the different species in disease.</p>
List of appendix cystic fibrosis and CBAVD research paper
<p>This is the dataset list of cystic fibrosis inside sperm wioth with <strong>congenital</strong> bilateral absence of the <strong>vas</strong> <strong>deferens (CBAVD)</strong></p>
Data and statistical analysis for: Response to Elexacaftor/Tezacaftor/Ivacaftor in intestinal organoids derived from patients with cystic fibrosis
<p>Data and code to reproduce all statistical analyses and figure in the manuscript "Response to Elexacaftor/Tezacaftor/Ivacaftor in intestinal organoids derived from patients with cystic fibrosis".</p> <p>The dataset is stored in the file `area_data.csv`, the columns are:</p> <ul> <li>`well` The ID of the well within the plate the measurement was taken</li> <li>`time` time from start of measurement</li> <li>`area` area of the organoids</li> <li>`patient` Anonymized ID of the patient</li> <li>`date` Date when the experiment was performed (date and patient ID identify the plate)</li> <li>`filename` The filename in raw data (not published) this plate was stored in. (uniquely identifies a plate)</li> <li>`mix` Identifies one od two variants of plate layouts used</li> <li>`replicate` ID of technical replicate (two technical replicates were done for each condition on the same plate)</li> <li>`type` The main condition - one of "TEZ/IVA", "ELX/TEZ/IVA", "FskOnly" (Control, only forskolin)</li> <li>`fsk_concentration` concentration of forskolin (μM)</li> </ul> <p>The file "organoids.Rmd" reproduces the analyses, other files are supporting to let the main analysis run.</p> <p>The analysis is written in R markdown.</p> <p> </p>
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Allen Brain Atlas
Allen Brain Atlas is an Allen Institute collection of brain map atlases, datasets, APIs, and analysis tools covering mouse, human, and non-human primate brain resources.
Annotated Behaviour and Observability Dataset (ABODe)
ABODe is a University of Edinburgh DataShare dataset for behavior classification in group-housed mice using home-cage video, identities, bounding boxes, ground-plate positions, and annotator labels.
DANDI Archive for NWB datasets
DANDI is a BRAIN Initiative archive for publishing and sharing neurophysiology data, including electrophysiology, optophysiology, and behavioral data packaged as NWB and related standards.
International Brain Laboratory public data
The International Brain Laboratory public data releases expose standardized mouse decision-making experiments, including Neuropixels recordings, widefield calcium imaging, behavior, and session metadata accessed through the ONE API.
OpenNeuro
OpenNeuro is a free, open platform for sharing neuroimaging datasets, with public search, dataset pages, and download paths for web, S3, DataLad, and the OpenNeuro CLI.