Keywords: فیبروز سیستیک; AHI, apnoea/hypopnoea index; BMI, body mass index; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane condutance regulator; ERS, European Respiratory Society; FEF25–75, forced expiratory flow between 25% and 75% of maximal expiratory flow; FEV1, for
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Keywords: فیبروز سیستیک; Chronic pancreatitis; Fatty acid profile; Discriminant analysis; Diabetes mellitus; MalnutritionAP, acute pancreatitis; BMI, body mass index; CF, cystic fibrosis; CON, healthy controls; CP, chronic pancreatitis; CRP, C-reactive protein; CT, computer tomog
Keywords: فیبروز سیستیک; AAD, Adaptive Aerosol Delivery; CF, cystic fibrosis; CMS, colistimethate sodium; DD, delivered dose; DDR, drug delivery rate; DPI, dry powder inhaler; D(v,50), median volume diameter; GSD, Geometric Standard Deviation; MIU, million international units; MM
Keywords: فیبروز سیستیک; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane conductance regulator; CPET, cardiopulmonary exercise test; Db10, potentiated doublets at 10 Hz; Db100, potentiated doublets at 100 Hz; Db10:100, ratio between potentiated doublets at 10 Hz and 100
Keywords: فیبروز سیستیک; CF, cystic fibrosis; CFU, colony forming unit; CHI, chitosan; COPD, chronic obstructive pulmonary diseases; CUR, curcumin; dA, aerodynamic diameter; dG, geometric diameter; DMEM, Dulbecco’s modified Eagle’s medium; DMSO, dimethyl sulfoxide; DPI, dry powde
Keywords: فیبروز سیستیک; Thymus vulgaris; Anti-inflammatory; Normal bronchial and tracheal epithelial cells (HBEpC/HTEpC); Lung cancer; Thymol; NF-κBAbs, Antibodies; HBEpC/HTEpC, Bronchial/tracheal epithelial cells; CF, cystic fibrosis; COPD, chronic obstructive pulmonary disease
Keywords: فیبروز سیستیک; AGP, α-1-acid glycoprotein; apoD, apolipoprotein D; CF, cystic fibrosis; CMPF, 3-carboxy-4-methyl-5-propyl-2-furanpropanoic acid; DIS, di-iodosalicylic acid; FA, fatty acid; FABP, fatty acid-binding protein; Gd, glycodelin; HDL, high density lipoproteins;
Keywords: فیبروز سیستیک; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane receptor; QNS, quantity not sufficient; CLSI, Clinical and Laboratory Standards Institute.Sweat chloride; Sweat rate; Cystic fibrosis; Biological variability; Limit of quantitation
Keywords: فیبروز سیستیک; CF, cystic fibrosis; CO2, carbon dioxide; CV, coefficient of variation; FEV1, forced expiratory volume in one second; FVC, forced vital capacity; FRC, functional residual capacity; ITGV, intrathoracic gas volume; LCI, lung clearance index; MEF50, maximum
Keywords: فیبروز سیستیک; CDs, cyclodextrins; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane regulator; TER, transepithelial electrical resistance; TJs, tight junctionsBronchial epithelium; Cyclodextrin; Curcumin; Epithelial integrity; TER; VA10
Keywords: فیبروز سیستیک; CF, cystic fibrosis; PA, Pseudomonas aeruginosa; HPA, hypermutable Pseudomonas aeruginosa; MDR, multi-drug resistant; FEV1%, forced expiratory volume in 1 s; MEF 25–75%, maximal mid-expiratory flow.Cystic fibrosis; Hypermutable; Pseudomonas aeruginosa
Keywords: فیبروز سیستیک; BMI, Body mass index; BMIp, BMI percentile; CDC, Centers for Disease Control and Prevention; CF, Cystic fibrosis; CFF, CF Foundation; FEV1p, Percent predicted forced expiratory volume in 1 second; LFA, Length-for-age; LFAp, LFA percentile; MI, Meconium il
Keywords: فیبروز سیستیک; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane conductance regulator; (ΔIeq-sc), equivalent short-circuit currents; ENaC, epithelial Na+ channel; Fsk, forskolin; Gen, Genistein; HBE (cells), human bronchial epithelial cells; SEM, standard error
Keywords: فیبروز سیستیک; Ab-PAS, Alcian blue-Periodic Acid Schiff; CF, cystic fibrosis; CFTR, Cystic Fibrosis Transmembrane conductance Regulator; COPD, chronic obstructive pulmonary disease; DIOS, distal intestinal obstruction syndrome; EDTA, 2,2ʹ,2ʺ,2‴-(Ethane-1,2-diyldinitrilo
Keywords: فیبروز سیستیک; ACFBAL, Australasian Cystic Fibrosis BronchoAlveolar Lavage; BAL, bronchoalveolar lavage; CF, cystic fibrosis; CFU, colony forming units; CI, confidence interval; ERIC, enterobacterial repetitive intergenic consensus; IQR, interquartile range; MLST, multi
Keywords: فیبروز سیستیک; CF, Cystic Fibrosis; PH, pulmonary hypertension; FEV1, Forced Expiratory Volume in one second; EF, ejection fraction; TAPSE, tricuspid annular plane systolic excursion; PAP, pulmonary artery pressure; hs-CRP, highly sensitive C-reactive protein; ESR, Eryt
Keywords: فیبروز سیستیک; BAL, bronchoalveolar lavage; BALF, bronchoalveolar lavage fluid; BOS, bronchiolitis obliterans syndrome; CF, Cystic Fibrosis; CRS, chronic rhinosinusitis; CTSS, cathepsin S; ECM, extracellular matrix; IFN, interferon; IL, interleukin; LAW, lower airways;
Keywords: فیبروز سیستیک; CFTR; Cystic fibrosis; Nucleotide binding domains; ΔF508; G551D; Molecular dynamicsATP, adenosine triphosphate; ABC, ATP-binding cassette; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane conductance regulator; EM, electron microscopy; ICL, intrac
Keywords: فیبروز سیستیک; IFN-α, interferon-alpha; dsRNA, double stranded RNA; CF, cystic fibrosis; IHC, immunohistochemistry; qRT-PCR, quantitative real-time polymerase chain reaction; GAPDH, glyceraldehyde 3-phosphate dehydrogenase; VP, viral protein; ISG, interferon-stimulated
Keywords: فیبروز سیستیک; AMI, acute mesenteric ischemia; CF, cystic fibrosis; CRF, corticotropin-releasing factor; ENS, enteric nervous system; FAP, familial adenomatous polyposis; GLP-2, glucagon-like peptide 2; IESCs, intestinal epithelial stem cell; NEC, neonatal necrotizing e
Keywords: فیبروز سیستیک; BMI, body mass index; CF, cystic fibrosis; CFRD, CF-related diabetes; FEV1, forced expiratory volume in one second; FVC, forced vital capacity; HbA1c, glycated haemoglobin; INR, international normalized ratio; Zn, zincZinc; Cystic fibrosis; Retrospective
Keywords: فیبروز سیستیک; AMR, antibody mediated rejection; BAL, bronchoalveolar lavage; BOS, bronchiolitis obliterans syndrome; CDC, complement-dependent cytotoxicity; CF, cystic fibrosis; CLAD, chronic lung allograft dysfunction; CMV, cytomegalovirus; CT, computed tomography; DA
Keywords: فیبروز سیستیک; ALP, alkaline phosphatase; ALT, alanine aminotransferase; AST, aspartate aminotransferase; CF, cystic fibrosis; CFLD, cystic fibrosis-associated liver disease; CFTR, cystic fibrosis conductance regulator; GGT, γ-glutamyltransferase; HSC, hepatic stellate
Keywords: فیبروز سیستیک; BMI, body mass index; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane regulator; ELISA, enzyme linked immunosorbent assay; FEV1, forced expiratory volume in one second; Ig, immunoglobulin; S–K, Shwachman–KulczyckiCystic fibrosis; Pneumococcus; Va
Keywords: فیبروز سیستیک; AUC, area under the curve; AUC-NO, area under the curve of the nitric oxide concentration/exhalation time plot; Cano, peripheral/small airway/alveolar nitric oxide concentration; CF, cystic fibrosis; Feno, fraction of exhaled nitric oxide; J’awno, large/c
Keywords: فیبروز سیستیک; CF, cystic fibrosis; BMIp, Body Mass Index percentile; %FEV1, percentage of predicted forced expiratory volume in 1 sSpiritual coping; Psychology; Long-term outcomes
Keywords: فیبروز سیستیک; TB, tuberculosis; Ig, immunoglobulins; NTM, nontuberculous mycobacteria; US, United States; MAC, Mycobacterium avium complex; COPD, chronic obstructive pulmonary disease; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane conductance regulator; HLA,
Keywords: فیبروز سیستیک; BDI, Beck Depression Inventory; CF, cystic fibrosis; DUMC, Duke University Medical Center; FSRP, Framingham Stroke Risk Profile; HR, hazard ratio; INSPIRE, Investigational Study of Psychological Intervention in Recipients of Lung Transplant; IRB, institut
Keywords: فیبروز سیستیک; CF, cystic fibrosis; Pa, Pseudomonas aeruginosa; OP, oropharyngeal; BAL, bronchoalveolar lavage; EPIC OBS, Early Pseudomonas Infection Control Observational Study; CFFNPR, CFF National Patient Registry; GEE, generalized estimating equations; ROC, receiver
Keywords: فیبروز سیستیک; CF, cystic fibrosis; ELISA, enzyme linked immuno assay; P. aeruginosa, Pseudomonas aeruginosa; FEV1, forced expiratory volume in one secondCystic fibrosis; Pseudomonas aeruginosa; Serum antibodies; Prognostic value
Keywords: فیبروز سیستیک; Microbiome; Probiotics; Microbiota; Airways; Intestinal tract; Chronic lung diseaseBOS, bronchiolitis obliterans syndrome; CF, cystic fibrosis; COPD, chronic obstructive pulmonary disease; FMT, fecal microbiota transplantation; IBD, inflammatory bowel dis
Keywords: فیبروز سیستیک; CF, Cystic fibrosis; BAL, Bronchoalveolar lavage; ELISA, Enzyme-linked immunosorbent assay; CIE, Crossed immune electrophoresis; RIA, Radioimmunoassay; ExoA, Exotoxin A; ELA, Elastase; AP, Alkaline protease; TTSS, Type three secretion system; PCR, Polymer
Keywords: فیبروز سیستیک; ANOVA, analysis of variance; CE, capillary electrophoresis; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane conductance regulator; Da, Dalton; DAD, diode array detector; DCD, dermcidin; EI, electron impact ionization; EIA, enzyme immunoassay; ELI
Keywords: فیبروز سیستیک; Randomized controlled trials; Real life studies; Asthma; COPDRCTs, randomized controlled trials; COPD, chronic obstructive pulmonary disease; CAP, community acquired pneumonia; CF, cystic fibrosis; ITT, intention-to-treat; ICSs, inhaled corticosteroids; B
Keywords: فیبروز سیستیک; CDC, Centers for Disease Control and Prevention; CF, cystic fibrosis; CTSA, Clinical and Translational Science Award; EGAPP, Evaluation of Genomic Applications in Practice and Prevention; FHH, family health history; GEP, gene expression profiling; NIH, Na
Keywords: فیبروز سیستیک; ARE, acute respiratory exacerbation; CF, cystic fibrosis; CSLD, chronic suppurative lung disease; HRCT, high resolution CT
Keywords: فیبروز سیستیک; BSA, Bovine serum albumin; CF, Cystic fibrosis; CFD, Computational fluid dynamics; CLIJ, Confined liquid impinging jets; COPD, Chronic obstructive pulmonary disorder; dae, Aerodynamic diameter; DEM, Discrete element method; DPI, Dry powder inhaler; FPF, F
Keywords: فیبروز سیستیک; VLP, virus-like particle; CF, cystic fibrosis; TTV, Torque Teno virus; CD, Crohn's diseasebacteriophage; virus; microbiome; virome; metagenome
Keywords: فیبروز سیستیک; EQA, external quality assessment;; ST, sweat test; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane regulator; ISS, Istituto Superiore di Sanità; CNMR, National Centre for Rare Diseases; SIFC, Italian Cystic Fibrosis Society; SIMMESN, Italian Soci
Keywords: فیبروز سیستیک; CAP, community-acquired pneumonia; ICU, intensive care unit; RCTs, randomized controlled trials; ARDS, acute respiratory distress syndrome; CF, cystic fibrosis; COPD, chronic obstructive pulmonary disease; Pao2/Fio2, ratio of partial pressure arterial oxy
Keywords: فیبروز سیستیک; Cardiac output; Stroke volume; CFTR; Cardiopulmonary exercise test; Exercise capacity; Functional limitation; Pulmonary functionBSA, body surface area; Ca2+, calcium; Ca-vO2, arterio-venous oxygen content difference; CF, cystic fibrosis; CFTR, cystic fibr
Keywords: فیبروز سیستیک; Akt, serine/threonine-specific protein kinase; AMD, age-related macular degeneration; CF, cystic fibrosis; COPD, chronic obstructive pulmonary disease; c-Src, proto-oncogene tyrosine-protein kinase; EC, endothelial cell; FGF-2, fibroblast growth factor-2;
Keywords: فیبروز سیستیک; Aspergillosis; Lung infections; Computed tomographyA. fumigatus, Aspergillus fumigatus; ABPA, allergic broncho-pulmonary aspergillosis; COPD, chronic obstructive pulmonary disease; CF, cystic fibrosis; HRCT, high-resolution computed tomography; CAN, chron
Effect of ivacaftor in patients with advanced cystic fibrosis and a G551D-CFTR mutation: Safety and efficacy in an expanded access program in the United States
Keywords: فیبروز سیستیک; AE, Adverse Event; SAE, Serious Adverse Event; CF, Cystic Fibrosis; CFF, Cystic Fibrosis Foundation; CFTR, Cystic Fibrosis Transmembrane Conductance Regulator; FDA, Federal Drug Administration; FEV1, Forced expiratory volume in 1 sCystic fibrosis (CF); Cy
The phospholipid flippase ATP8B1 mediates apical localization of the cystic fibrosis transmembrane regulator
Keywords: فیبروز سیستیک; PFIC1, progressive familial intrahepatic cholestasis type 1; CFTR, cystic fibrosis transmembrane conductance regulator; CF, cystic fibrosis; Isc, short circuit current; TGN, trans-Golgi network; ASBT, apical sodium-dependent bile acid transporter; AP, alk
Epithelial sodium channel (ENaC) family: Phylogeny, structure–function, tissue distribution, and associated inherited diseases
Keywords: فیبروز سیستیک; ASIC, acid-sensing ion channel; ASL, airway surface liquid; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane conductance regulator; ENaC, epithelial sodium channel; ECF, extracellular fluid; ICF, intracellular fluid; PHA, pseudohypoaldosteronism;
Improved Clinical and Radiographic Outcomes After Treatment With Ivacaftor in a Young Adult With Cystic Fibrosis With the P67L CFTR Mutation
Keywords: فیبروز سیستیک; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane conductance regulator
Electronic monitoring reveals highly variable adherence patterns in patients prescribed ivacaftor ★
Keywords: فیبروز سیستیک; BMI, body mass index; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane conductance regulator; EM, electronic monitoring; FEV1PP, forced expiratory volume in 1 second percent predicted; MEMS, Medication Events Monitoring System; MPR, medication pos
Identification of outer membrane Porin D as a vitronectin-binding factor in cystic fibrosis clinical isolates of Pseudomonas aeruginosa
Keywords: فیبروز سیستیک; aa, amino acid; BSA, bovine serum albumin; CF, cystic fibrosis; COPD, chronic obstructive pulmonary disease; CPM, counts per minute; DBA, direct binding assay; ECM, extracellular matrix; FCS, fetal calf serum; HBD, heparin binding domain; IPTG, isopropyl-
Should diffuse bronchiectasis still be considered a CFTR-related disorder?
Keywords: فیبروز سیستیک; CFTR, cystic fibrosis transmembrane conductance regulator; CF, cystic fibrosis; CFTR-RD, CFTR-related disorder; DB, diffuse bronchiectasis; WT, wild-type; IQR, interquartile range; M, mild; UV, unclassified variant; P, rare polymorphism; NNV, non-neutral