Alternative outcomes for the multiple breath washout in children with CF
Keywords: فیبروز کیستیک; Cystic fibrosis; Multiple breath washout; Clinical trials;
مقالات ISI فیبروز کیستیک (ترجمه نشده)
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Survival benefit of induction immunosuppression in cystic fibrosis lung transplant recipientsâ
Keywords: فیبروز کیستیک; Cystic fibrosis; Induction; Immunosuppression; Lung transplantation; Survival;
Proximity to blue spaces and risk of infection with Pseudomonas aeruginosa in cystic fibrosis: A case-control analysisâ
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Keywords: فیبروز کیستیک; Cystic fibrosis; Water; Pseudomonas aeruginosa;
Strain rate echocardiography uncovers subclinical left ventricular dysfunction in cystic fibrosis
Keywords: فیبروز کیستیک; Cystic fibrosis; Left ventricle; Heart function; Echocardiography; Strain imaging
Safety and efficacy of Creon® Micro in children with exocrine pancreatic insufficiency due to cystic fibrosis
Keywords: فیبروز کیستیک; Clinical trial; Creon; Cystic fibrosis; Pancreatic enzyme replacement therapy; Exocrine pancreatic insufficiency; Pancreatin;
Cystic Fibrosis Screen Positive, Inconclusive Diagnosis (CFSPID): A new designation and management recommendations for infants with an inconclusive diagnosis following newborn screening
Keywords: فیبروز کیستیک; Cystic fibrosis; Newborn screening; CRMS; CFSPID;
Absence bilatérale des canaux déférents : du diagnostic à l’assistance médicale à la procréation. Expérience de 3 centres
Keywords: فیبروز کیستیک; Absence bilatérale des canaux déférents; Mucoviscidose; Biopsie testiculaire; Aspiration épididymaire; Assistance médicale à la procréationCongenital bilateral absence of vas deferens; Cystic fibrosis; Testicular biopsy; Epididymal aspiration; Assisted re
A phase 3, open-label, randomized trial to evaluate the safety and efficacy of levofloxacin inhalation solution (APT-1026) versus tobramycin inhalation solution in stable cystic fibrosis patients
Keywords: فیبروز کیستیک; Cystic fibrosis; Antibiotics; Pseudomonas; Aerosol; Fluoroquinolone;
Molecular epidemiology of Aspergillus collected from cystic fibrosis patients
Keywords: فیبروز کیستیک; Cystic fibrosis; Aspergillus; Cryptic species;
Randomized, single blind, controlled trial of inhaled glutathione vs placebo in patients with cystic fibrosis
Keywords: فیبروز کیستیک; CF; cystic fibrosis; GSH; glutathione; CFTR; cystic fibrosis transmembrane regulator; FEV1; forced expiratory volume in 1Â s; FVC; forced vital capacity; FEF25-75; flow at the mid-portion of forced vital capacity; 6MWT; six minute walking test; BMI; bod
Asociación entre el cambio relativo de peso e índice de masa corporal con la función pulmonar en adolescentes y adultos con fibrosis quística: influencia del género y la diabetes
Keywords: فیبروز کیستیک; Diabetes; Fibrosis quística; Función pulmonar; Estado nutricionalDiabetes; Cystic fibrosis; Lung function; Nutritional status
Mechanism for glutathione-mediated protection against the Pseudomonas aeruginosa redox toxin, pyocyanin
Keywords: فیبروز کیستیک; Pseudomonas aeruginosa; Pyocyanin; Glutathione; Redox chemistry; Wounds; Cystic fibrosis
Concentration of fractional excretion of nitric oxide (FENO): A potential airway biomarker of restored CFTR function
Keywords: فیبروز کیستیک; ATS; American Thoracic Society; BMI; body mass index; CF; cystic fibrosis; CFTR; cystic fibrosis transmembrane conductance regulator; CI; confidence interval; COPD; chronic obstructive pulmonary disease; FENO; fractional excretion of nitric oxide; FEV1; f
Function, pharmacological correction and maturation of new Indian CFTR gene mutations
Keywords: فیبروز کیستیک; CBAVD; congenital bilateral absence of the vas deferens; CF; cystic fibrosis; CFTR; cystic fibrosis transmembrane conductance regulator; GFP; green fluorescent protein; WT-CFTR; wild-type CFTR; Missense CF mutations; India; L69H-CFTR; S549N-CFTR; Low temp
Polyunsaturated fatty acid supplementation reverses cystic fibrosis-related fatty acid abnormalities in CFTR−/− mice by suppressing fatty acid desaturases
Keywords: فیبروز کیستیک; Cystic fibrosis; Fatty acid; Desaturase; Docosahexaenoate; Eicosapentaenoate; Arachidonate
Evidence against resveratrol as a viable therapy for the rescue of defective ÎF508 CFTR
Keywords: فیبروز کیستیک; CF; cystic fibrosis; CFTR; cystic fibrosis transmembrane conductance regulator; DMEM; Dulbecco's modified Eagle Medium; ENaC; epithelial sodium channel; IBMX; isobutyl-methyl xanthine; SDS-PAGE; sodium dodecylsulphate polyacrylamide gel electrophoresis; W
Children with Down Syndrome Are High-Risk for Severe Respiratory Syncytial Virus Disease
Keywords: فیبروز کیستیک; CAA; Congenital airway anomaly; CF; Cystic fibrosis; CHD; Congenital heart disease; CLD; Chronic lung disease; HR; Hazard ratio; HSHD; Hemodynamically significant heart disease; ICD-9-CM; International Classification of Diseases, 9th revision, Clinical Mo
Physical Exercise Recommendations Improve Postural Changes Found in Children and Adolescents with Cystic Fibrosis: A Randomized Controlled Trial
Keywords: فیبروز کیستیک; CF; Cystic fibrosis; ES; Effect size; FEF25%-75%; Forced expiratory flow between 25% and 75% of FVC; FEV1; Forced expiratory volume in the first second; FVC; Forced vital capacity; HSL-PUCRS; Hospital São Lucas, PontÃficia Universidade Católica do Rio
Determination of ciprofloxacin and levofloxacin in human sputum collected from cystic fibrosis patients using microextraction by packed sorbent-high performance liquid chromatography photodiode array detector
Keywords: فیبروز کیستیک; MEPS–HPLC–PDA; Method development; Levofloxacin and ciprofloxacin; Sputum; Sample preparation; Cystic fibrosis
Cystic fibrosis and the role of gastrointestinal outcome measures in the new era of therapeutic CFTR modulation
Keywords: فیبروز کیستیک; Cystic fibrosis; Outcome measures; End points; Gastrointestinal; Clinical trials; Intestinal pH; Intestinal transit time; Bile acid metabolism; Intestinal inflammation; Exocrine pancreatic insufficiency; Fat malabsorption;
Long-term treatment with oral N-acetylcysteine: Affects lung function but not sputum inflammation in cystic fibrosis subjects. A phase II randomized placebo-controlled trial
Keywords: فیبروز کیستیک; Cystic fibrosis; Clinical trial; Anti-oxidant; Glutathione; Lung function;
Capillary electromigration techniques as tools for assessing the status of vitamins A, C and E in patients with cystic fibrosis
Keywords: فیبروز کیستیک; Capillary electrophoresis; Cystic fibrosis; Vitamin A; Vitamin C; Vitamin E;
Enhancement of premature stop codon readthrough in the CFTR gene by Ataluren (PTC124) derivatives
Keywords: فیبروز کیستیک; Cystic fibrosis; PTCs readthrough; CFTR gene; Green fluorescent protein; Nonsense mutation; Fluorinated oxadiazole;
Overcoming barriers in Pseudomonas aeruginosa lung infections: Engineered nanoparticles for local delivery of a cationic antimicrobial peptide
Keywords: فیبروز کیستیک; Nanoparticles; PLGA; Antimicrobial peptide; Pulmonary delivery; Pseudomonas aeruginosa; Biofilm; Cystic fibrosis
The hsa-miR-125a/hsa-let-7e/hsa-miR-99b cluster is potentially implicated in Cystic Fibrosis pathogenesis
Keywords: فیبروز کیستیک; Cystic Fibrosis; MicroRNAs; CFTR; SNPs
High frequency hearing thresholds and product distortion otoacoustic emissions in cystic fibrosis patients
Keywords: فیبروز کیستیک; Cystic fibrosis; Aminoglycosides; AudiometryFibrose cística; Aminoglicosídeos; Audiometria
Resistencia insulínica, disfunción de la célula β pancreática y diferencias en los puntos intermedios de las curvas de glucemia e insulina tras una sobrecarga oral estándar de glucosa en adultos con fibrosis quística
Keywords: فیبروز کیستیک; Diabetes; Fibrosis quística; Resistencia a la insulina; Función de célula β; Test de tolerancia oral a la glucosa a las 2 horasDiabetes; Cystic fibrosis; Insulin resistance; β-cell function; 2-hour oral glucose tolerance test
Insulin resistance, β-cell dysfunction and differences in curves of plasma glucose and insulin in the intermediate points of the standard glucose tolerance test in adults with cystic fibrosis
Keywords: فیبروز کیستیک; Diabetes; Cystic fibrosis; Insulin resistance; β-cell function; 2-h oral glucose tolerance testDiabetes; Fibrosis quística; Resistencia a la insulina; Función de célula β; Test de tolerancia oral a la glucosa a las 2 horas
Intrapatient diversity of Achromobacter spp. involved in chronic colonization of Cystic Fibrosis airways
Keywords: فیبروز کیستیک; Achromobacter; Cystic Fibrosis; Colonization; Diversity; Genome; Adaptation;
Upper aero-digestive contamination by Pseudomonas aeruginosa and implications in Cystic Fibrosis
Keywords: فیبروز کیستیک; Pseudomonas aeruginosa; Cystic Fibrosis; Mouth; Paranasal sinuses; Nasal cavity;
Targeting glycosaminoglycans in the lung by an engineered CXCL8 as a novel therapeutic approach to lung inflammation
Keywords: فیبروز کیستیک; BAL; brochoalveolar lavage; COPD; chronic obstructive pulmonary disease; CF; cystic fibrosis; GAG; glycosaminoglycan; GM-CSF; granulocyte macrophage colony-stimulating factor; HMVEC-L; human lung microvascular endothelial cells; KC; keratinocytes-derived
Fibrosis quÃstica: detección bioquÃmica y diagnóstico molecular
Keywords: فیبروز کیستیک; Fibrosis quÃstica; Diagnóstico molecular; Cribado neonatal, Gen CFTR; Cystic fibrosis; Molecular diagnosis; Newborn screening, CFTR gene;
Transcriptome meta-analysis reveals common differential and global gene expression profiles in cystic fibrosis and other respiratory disorders and identifies CFTR regulators
Keywords: فیبروز کیستیک; Cystic fibrosis; respiratory disease; differentiation, transcriptome; meta-analysis
Denitrification by cystic fibrosis pathogens - Stenotrophomonas maltophilia is dormant in sputum
Keywords: فیبروز کیستیک; Cystic fibrosis; Pseudomonas aeruginosa; Achromobacter xylosoxidans; Burkholderia multivorans and Stenotrophomonas maltophilia; N2O; Denitrification;
Persistent cystic fibrosis isolate Pseudomonas aeruginosa strain RP73 exhibits an under-acylated LPS structure responsible of its low inflammatory activity
Keywords: فیبروز کیستیک; Pseudomonas aeruginosa; Cystic fibrosis; Lipopolysaccharide; Lipid A; Chronic infections in cystic fibrosis; Structural analysis
Vitamin D deficiency is associated with pulmonary dysfunction in cystic fibrosis
Keywords: فیبروز کیستیک; BMI; body mass index; CF; cystic fibrosis; CFRD; cystic fibrosis-related diabetes; COPD; chronic obstructive pulmonary disease; DEXA; dual-energy X-ray absorptiometry; FEF25-75; forced expiratory flow at 25%-75% of forced vital capacity; FEV1; forced
Cystic fibrosis-related bone disease in children: Examination of peripheral quantitative computed tomography (pQCT) data
Keywords: فیبروز کیستیک; Cystic fibrosis; Bone; Muscle; Bone strength; pQCT; Volumetric bone mineral density
The Cholangiopathies
Keywords: فیبروز کیستیک; ADPLD; autosomal dominant polycystic liver disease; ADPKD; autosomal dominant polycystic kidney disease; AIDS; acquired immunodeficiency syndrome; ARPKD; autosomal recessive polycystic kidney disease; cAMP; cyclic adenosine monophosphate; CF; cystic fibro
Osteopontin is increased in cystic fibrosis and can skew the functional balance between ELR-positive and ELR-negative CXC-chemokines
Keywords: فیبروز کیستیک; Cystic fibrosis; Host defense; Osteopontin; Chemokines; Neutrophils;
The low PLC-δ1 expression in cystic fibrosis bronchial epithelial cells induces upregulation of TRPV6 channel activity
Keywords: فیبروز کیستیک; TRPV6; Cystic fibrosis; Human bronchial epithelial cells; CalciumCF, Cystic Fibrosis; CFTR, Cystic Fibrosis Transmembrane conductance Regulator; hAEC, primary human airway epithelial cells; PIP2, phosphatidylinositol 4,5-bisphosphate; PI-PLC, phosphoinosi
SERCA and PMCA pumps contribute to the deregulation of Ca2 + homeostasis in human CF epithelial cells
Keywords: فیبروز کیستیک; F508del-CFTR; PMCA; SERCA; Cystic fibrosis; Calcium signaling
Involvement of the heterodimeric interface region of the nucleotide binding domain-2 (NBD2) in the CFTR quaternary structure and membrane stability
Keywords: فیبروز کیستیک; CFTR; NBD2; Dimerization; Membrane stability; Quaternary structure; Cystic fibrosis;
Loss of cftr function leads to pancreatic destruction in larval zebrafish
Keywords: فیبروز کیستیک; Cftr; Cystic fibrosis; Pancreas
Aggregates of mutant CFTR fragments in airway epithelial cells of CF lungs: New pathologic observations
Keywords: فیبروز کیستیک; Cystic fibrosis; Mutant CFTR; Lung epithelium;
Identification of Scedosporium boydii catalase A1 gene, a reactive oxygen species detoxification factor highly expressed in response to oxidative stress and phagocytic cells
Keywords: فیبروز کیستیک; Catalase; Cystic fibrosis; Phagocytes; ROS; Scedosporium apiospermum complex; SOD
Variants in Solute Carrier SLC26A9 Modify Prenatal Exocrine Pancreatic Damage in Cystic Fibrosis
Keywords: فیبروز کیستیک; 660W; Illumina 660W-Quad BeadChip; CF; Cystic fibrosis; CFTR; Cystic fibrosis transmembrane conductance regulator; IRT; Immunoreactive trypsinogen; NBS; Newborn screening/screened; Omni5; Illumina Omni5-Quad BeadChip; QC; Quality control; SNP; Single nucl
Ãtude in vitro de la durée d'écoulement de solutions antibiotiques préparées dans des diffuseurs portables élastomériques : effet du stockage au froid pendant trois à sept jours
Keywords: فیبروز کیستیک; Diffuseur portable élastomérique; Antibiotique; Mucoviscidose; Traitement à domicile; Durée de perfusion; Réfrigération; Elastomeric infusion device; Antibiotic; Cystic fibrosis; Home care; Duration of infusion; Cold storage;
Diversité physiopathologique du microbiote respiratoire
Keywords: فیبروز کیستیک; Microbiote respiratoire; dysmicrobisme; dysbiose; infection respiratoire aiguë; pneumopathie; pathologies respiratoires chroniques; mucoviscidose; BPCO; asthme; bactéries pathogènes opportunistes; Airway microbiota; dysbiosis; acute respiratory tract i
Preclinical safety and efficacy models for pulmonary drug delivery of antimicrobials with focus on in vitro models
Keywords: فیبروز کیستیک; Anti-infectives; Tuberculosis; Pneumonia; Cystic fibrosis; Cell culture models; Disease-relevant models; Alternatives to animal testing; 3R principle; Calu-3; A549;
Nanocomplexes for gene therapy of respiratory diseases: Targeting and overcoming the mucus barrier
Keywords: فیبروز کیستیک; Asthma; Cystic fibrosis; Lung cancer; Mucolytics; Poly(ethylene glycol); SputumPolyethyleinimine (PubMed CID: 9033); Poly-l-lysine (PubMed CID: 53628747); Ethylene glycol (PubMed CID: 174); Chitosan (PubMed CID: 71853); PAMAM G0 dendrimer (PubMed CID: 414