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Subject Areas on Research
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17beta-Estradiol inhibits Ca2+-dependent homeostasis of airway surface liquid volume in human cystic fibrosis airway epithelia.
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5' exon replacement and repair by spliceosome-mediated RNA trans-splicing.
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A cystic fibrosis transmembrane conductance regulator splice variant with partial penetrance associated with variable cystic fibrosis presentations.
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A matched comparison of adjustment in children with cystic fibrosis and psychiatrically referred and nonreferred children.
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A review of rectal biopsy.
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A revised airway epithelial hierarchy includes CFTR-expressing ionocytes.
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A yeast metal resistance protein similar to human cystic fibrosis transmembrane conductance regulator (CFTR) and multidrug resistance-associated protein.
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AAV exploits subcellular stress associated with inflammation, endoplasmic reticulum expansion, and misfolded proteins in models of cystic fibrosis.
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Abnormal bone turnover in cystic fibrosis adults.
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Acrodermatitis enteropathica-like eruption and food allergy.
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Acute renal failure in cystic fibrosis: association with inhaled tobramycin therapy.
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Adverse alterations in bone metabolism are associated with lung infection in adults with cystic fibrosis.
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Age of Pseudomonas aeruginosa acquisition and subsequent severity of cystic fibrosis lung disease.
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An approach for treating the hepatobiliary disease of cystic fibrosis by somatic gene transfer.
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Approach to eradication of initial Pseudomonas aeruginosa infection in children with cystic fibrosis.
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Association of Transition Readiness to Intentional Self-Regulation and Hopeful Future Expectations in Youth With Illness.
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Atrial fibrillation after pulmonary transplant.
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Barriers to adherence to cystic fibrosis infection control guidelines.
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Basal-like cells constitute the proliferating cell population in cystic fibrosis airways.
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Bcl-2 suppresses sarcoplasmic/endoplasmic reticulum Ca2+-ATPase expression in cystic fibrosis airways: role in oxidant-mediated cell death.
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Bilateral sequential lung and simultaneous pancreas transplant: a new approach for the recipient with cystic fibrosis.
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Biochemical and biophysical identification of cystic fibrosis transmembrane conductance regulator chloride channels as components of endocytic clathrin-coated vesicles.
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Bronchiectasis: Mechanisms and Imaging Clues of Associated Common and Uncommon Diseases.
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Burden of influenza-related hospitalizations among children with sickle cell disease.
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CFTR as a cAMP-dependent regulator of sodium channels.
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CFTR haplotype analysis reveals genetic heterogeneity in the etiology of congenital bilateral aplasia of the vas deferens.
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CFTR: development of high- affinity antibodies and localization in sweat gland.
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CHEER National Study of Chronic Rhinosinusitis Practice Patterns: Disease Comorbidities and Factors Associated with Surgery.
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Case 6. Abnormal facies and mental retardation in a patient with cystic fibrosis of the pancreas.
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Cellular and viral splicing factors can modify the splicing pattern of CFTR transcripts carrying splicing mutations.
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Change over a 12-month period in the psychological adjustment of children and adolescents with cystic fibrosis.
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Characterization of the cystic fibrosis transmembrane conductance regulator in a colonocyte cell line.
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Children with Chronic Lung Disease: Facilitating Smoking Cessation for their Caregivers.
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Combined lung-liver-pancreas transplantation in a recipient with cystic fibrosis.
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Comparative efficacy and safety of 4 randomized regimens to treat early Pseudomonas aeruginosa infection in children with cystic fibrosis.
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Comparison of US Federal and Foundation Funding of Research for Sickle Cell Disease and Cystic Fibrosis and Factors Associated With Research Productivity.
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Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation.
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Coping and quality of life in patients awaiting lung transplantation.
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Correlation between sinus and lung cultures in lung transplant patients with cystic fibrosis.
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Cystic fibrosis and the National Institutes of Health consensus statement: are obstetrician-gynecologists ready to comply?
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Cystic fibrosis carrier screening using next generation sequencing: A cautionary tale.
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Cystic fibrosis gene mutations and pancreatitis risk: relation to epithelial ion transport and trypsin inhibitor gene mutations.
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Cystic fibrosis in Jews: frequency and mutation distribution.
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Cystic fibrosis in a black woman.
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Cystic fibrosis in the pancreas: recent advances provide new insights.
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Cystic fibrosis involving the cervix, mimicking a well-differentiated adenocarcinoma: a case report.
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Cystic fibrosis mutations and genetic predisposition to idiopathic chronic pancreatitis.
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Cystic fibrosis: combined hyperpolarized 3He-enhanced and conventional proton MR imaging in the lung--preliminary observations.
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Cystic fibrosis: usefulness of thoracic CT in the examination of patients before lung transplantation.
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Decreased Antibiotic Utilization After Sinus Surgery in Cystic Fibrosis Patients With Lung Transplantation.
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Defective innate immunity and hyperinflammation in newborn cystic fibrosis transmembrane conductance regulator-knockout ferret lungs.
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Desensitization to pancreatic enzyme intolerance in a child with cystic fibrosis.
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Developing Breakthrough Drugs for Heart Failure: Lessons Learned From the Cystic Fibrosis Experience.
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Diminished and erratic absorption of ergocalciferol in adult cystic fibrosis patients.
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Direct-from-blood RNA sequencing identifies the cause of post-bronchoscopy fever.
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Disruption of iron homeostasis in the lungs of transplant patients.
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Does the F508-CFTR mutation induce a proinflammatory response in human airway epithelial cells?
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Dynamic perfluorinated gas MRI reveals abnormal ventilation despite normal FEV1 in cystic fibrosis.
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Dysregulation of proteoglycan production by intrahepatic biliary epithelial cells bearing defective (delta-f508) cystic fibrosis transmembrane conductance regulator.
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Early anti-pseudomonal acquisition in young patients with cystic fibrosis: rationale and design of the EPIC clinical trial and observational study'.
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Effect of single vs bilateral lung transplantation on plasma surfactant protein D levels in idiopathic pulmonary fibrosis.
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Effects of GLP-1 and GIP on Islet Function in Glucose-Intolerant, Pancreatic-Insufficient Cystic Fibrosis.
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Efficacy of aerosolized tobramycin in patients with cystic fibrosis.
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Efficacy of alendronate in adults with cystic fibrosis with low bone density.
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Efficacy of pamidronate for osteoporosis in patients with cystic fibrosis following lung transplantation.
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Electron microprobe analysis of human labial gland secretory granules in cystic fibrosis.
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Endoscopic Sinus Surgery for Cystic Fibrosis: Variables Influencing Sinonasal and Pulmonary Outcomes.
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Enlarged Dural Sac in Idiopathic Bronchiectasis Implicates Heritable Connective Tissue Gene Variants.
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Epigenome editing of the CFTR-locus for treatment of cystic fibrosis.
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Evaluation of Antimicrobial Susceptibility Testing Methods for Burkholderia cenocepacia and Burkholderia multivorans Isolates from Cystic Fibrosis Patients.
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Expression of an abundant alternatively spliced form of the cystic fibrosis transmembrane conductance regulator (CFTR) gene is not associated with a cAMP-activated chloride conductance.
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Expression of the cystic fibrosis gene in adult human lung.
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Failure to thrive, diarrhea, cough, and oral candidiasis in a three-month-old boy.
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Fatal respiratory infection due to ST308 VIM-1-producing Pseudomonas aeruginosa in a lung transplant recipient: case report and review of the literature.
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Gastroesophageal reflux as a reversible cause of allograft dysfunction after lung transplantation.
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Gastrointestinal case of the day. Cystic fibrosis with colonic intussusception.
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Glucose tolerance, insulin secretion, and insulin sensitivity in children and adolescents with cystic fibrosis and no prior history of diabetes.
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Guidelines for Cystic Fibrosis Carrier Screening in the Prenatal/Preconception Period.
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Head and neck manifestations of cystic fibrosis and ciliary dyskinesia.
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Hepatic parenchymal cells in cystic fibrosis: ultrastructural evidence for abnormal intracellular transport.
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Homozygous sickle cell disease and cystic fibrosis in an adolescent.
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Hsp 70/Hsp 90 organizing protein as a nitrosylation target in cystic fibrosis therapy.
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Human cystic fibrosis transmembrane conductance regulator directed to respiratory epithelial cells of transgenic mice.
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Hypoxic Gene Expression of Donor Bronchi Linked to Airway Complications after Lung Transplantation.
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Idiopathic pancreatitis related to CFTR: complex inheritance and identification of a modifier gene.
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Illness specific patterns of psychological adjustment and cognitive adaptational processes in children with cystic fibrosis and sickle cell disease.
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Immunohistologic quantification of Pseudomonas aeruginosa in the tracheobronchial tree from patients with cystic fibrosis.
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Impact of Gene Patents and Licensing Practices on Access to Genetic Testing for Cystic Fibrosis
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Increased expression of senescence markers in cystic fibrosis airways.
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Increased risk of idiopathic chronic pancreatitis in cystic fibrosis carriers.
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Infections in patients with cystic fibrosis following lung transplantation.
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Inflammatory cell distribution within and along asthmatic airways.
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Initial Pseudomonas aeruginosa treatment failure is associated with exacerbations in cystic fibrosis.
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Injection laryngoplasty in children with cystic fibrosis and abnormal swallow.
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Inspissation of pancreatic zymogen material in cystic fibrosis.
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Internal consistency of DSM-III diagnoses using the symptom scales of the Child Assessment Schedule.
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International guidelines for the selection of lung transplant candidates: 2006 update--a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation.
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Iron accumulates in the lavage and explanted lungs of cystic fibrosis patients.
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Kaposi sarcoma after bilateral lung transplantation.
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Klotho Inhibits Interleukin-8 Secretion from Cystic Fibrosis Airway Epithelia.
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Localization of the cystic fibrosis transmembrane conductance regulator in human bile duct epithelial cells.
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Localization of the cystic fibrosis transmembrane conductance regulator in pancreas.
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Long-term efficacy and safety of 12 months of valganciclovir prophylaxis compared with 3 months after lung transplantation: a single-center, long-term follow-up analysis from a randomized, controlled cytomegalovirus prevention trial.
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Loss of cftr function leads to pancreatic destruction in larval zebrafish.
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Lung transplantation for cystic fibrosis patients with Burkholderia cepacia complex. Survival linked to genomovar type.
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Major and massive hemoptysis: reassessment of conservative management.
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Mapping and correcting hyperpolarized magnetization decay with radial keyhole imaging.
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Mapping strategies for multiple linked markers.
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Medical and obstetric complications among pregnant women with cystic fibrosis.
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Mild cystic fibrosis in a consanguineous family.
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Mild cystic fibrosis linked to chromosome 7q22 markers with an uncommon haplotype.
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Missense mutation (G480C) in the CFTR gene associated with protein mislocalization but normal chloride channel activity.
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Molecular basis of defective anion transport in L cells expressing recombinant forms of CFTR.
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Mucus concentration-dependent biophysical abnormalities unify submucosal gland and superficial airway dysfunction in cystic fibrosis.
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Multicenter characterization and validation of the intron-8 poly(T) tract (IVS8-T) status in 25 Coriell cell repository cystic fibrosis reference cell lines for cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation assays.
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Mutations of the cystic fibrosis gene and pancreatitis.
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Mycobacterium abscessus chest wall and pulmonary infection in a cystic fibrosis lung transplant recipient.
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Nasally inhaled dornase alfa in the postoperative management of chronic sinusitis due to cystic fibrosis.
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Novel human bronchial epithelial cell lines for cystic fibrosis research.
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Novel techniques for MR imaging of pulmonary airspaces.
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Opportunities to Improve Utilization of Palliative Care Among Adults With Cystic Fibrosis: A Systematic Review.
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Parenteral administration of oral medications in lung transplant recipients: An underrecognized problem.
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Partial correction of endogenous DeltaF508 CFTR in human cystic fibrosis airway epithelia by spliceosome-mediated RNA trans-splicing.
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Pharmacological modulation of the AKT/microRNA-199a-5p/CAV1 pathway ameliorates cystic fibrosis lung hyper-inflammation.
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Phosphorylation of the cystic fibrosis transmembrane conductance regulator.
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Physiology and pathophysiology of human airway mucus.
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Physiorack: an integrated MRI safe/conditional, gas delivery, respiratory gating, and subject monitoring solution for structural and functional assessments of pulmonary function.
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Preferences and Stated Adherence for Antibiotic Treatment of Cystic Fibrosis Pseudomonas Infections.
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Proteases and cystic fibrosis.
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Protein phosphorylation responses in normal and cystic fibrosis airway epithelial cell lines.
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Pseudomonas aeruginosa Leucine Aminopeptidase Influences Early Biofilm Composition and Structure via Vesicle-Associated Antibiofilm Activity.
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Pseudomonas aeruginosa serology and risk for re-isolation in the EPIC trial.
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Pseudomonas aeruginosa vesicles associate with and are internalized by human lung epithelial cells.
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Pseudomonas colonization in cystic fibrosis. A study of 160 patients.
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Pseudomonas invasion of type I pneumocytes is dependent on the expression and phosphorylation of caveolin-2.
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Psychological adjustment of children with cystic fibrosis: the role of child cognitive processes and maternal adjustment.
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Pulmonary embolization of microcrystalline cellulose in a lung transplant recipient.
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Purification of outer membrane vesicles from Pseudomonas aeruginosa and their activation of an IL-8 response.
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Reduced CFTR function and the pathobiology of idiopathic pancreatitis.
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Regulation of membrane chloride currents in rat bile duct epithelial cells.
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Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis.
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Repair of CFTR mRNA by spliceosome-mediated RNA trans-splicing.
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Resource use, costs, and utility estimates for patients with cystic fibrosis with mild impairment in lung function: analysis of data collected alongside a 48-week multicenter clinical trial.
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Respiratory syncytial virus infection enhances Pseudomonas aeruginosa biofilm growth through dysregulation of nutritional immunity.
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Respiratory therapies in the critical care setting. Should aerosolized antibiotics be administered to prevent or treat ventilator-associated pneumonia in patients who do not have cystic fibrosis?
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Roles of mucus adhesion and cohesion in cough clearance.
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SARS-CoV-2 Reverse Genetics Reveals a Variable Infection Gradient in the Respiratory Tract.
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Scanning electron microscopic study of the airways in normal children and in patients with cystic fibrosis and other lung diseases.
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Screening of CFTR mutations in an isolated population: identification of carriers and patients.
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Secretory Cells Dominate Airway CFTR Expression and Function in Human Airway Superficial Epithelia.
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Serum IgD concentrations in normal infants, children, and adults and in patients with elevated IgE.
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Short-term calcitriol administration improves calcium homeostasis in adults with cystic fibrosis.
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Significance of blood stream infection after lung transplantation: analysis in 176 consecutive patients.
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Simultaneous bilateral lung and pancreas transplantation in recipient with cystic fibrosis.
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Six-year molecular analysis of Burkholderia cepacia complex isolates among cystic fibrosis patients at a referral center for lung transplantation.
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Society for Maternal-Fetal Medicine Consult Series #57: Evaluation and management of isolated soft ultrasound markers for aneuploidy in the second trimester: (Replaces Consults #10, Single umbilical artery, October 2010; #16, Isolated echogenic bowel diagnosed on second-trimester ultrasound, August 2011; #17, Evaluation and management of isolated renal pelviectasis on second-trimester ultrasound, December 2011; #25, Isolated fetal choroid plexus cysts, April 2013; #27, Isolated echogenic intracardiac focus, August 2013).
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Splenorenal shunt with underlying cirrhosis of the liver.
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Stability and change in the psychological adjustment of mothers of children and adolescents with cystic fibrosis and sickle cell disease.
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Standard care versus protocol based therapy for new onset Pseudomonas aeruginosa in cystic fibrosis.
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Stress, coping, and family functioning in the psychological adjustment of mothers of children and adolescents with cystic fibrosis.
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Sublingual tacrolimus for immunosuppression in lung transplantation: a potentially important therapeutic option in cystic fibrosis.
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Submucosal glands are the predominant site of CFTR expression in the human bronchus.
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Survey of cystic fibrosis transmembrane conductance regulator genotypes in primary sclerosing cholangitis.
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Survival Benefit of Lung Transplantation in the Modern Era of Lung Allocation.
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Survival after lung transplantation of cystic fibrosis patients infected with Burkholderia cepacia complex.
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Survival difference between high-risk and low-risk CFTR genotypes after lung transplant.
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Survival of lung transplant patients with cystic fibrosis harboring panresistant bacteria other than Burkholderia cepacia, compared with patients harboring sensitive bacteria.
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The impact of cystic fibrosis and PSTI/SPINK1 gene mutations on susceptibility to chronic pancreatitis.
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The molecular basis of disease variability among cystic fibrosis patients carrying the 3849+10 kb C-->T mutation.
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The role of biomedical and psychosocial processes in the intellectual and academic functioning of children and adolescents with cystic fibrosis.
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The role of cystic fibrosis gene mutations in determining susceptibility to chronic pancreatitis.
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The wait for the waitlist: The next challenge in the lung allocation system.
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Trends in the clinical characteristics of the U.S. cystic fibrosis patient population from 1995 to 2005.
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Tyloxapol inhibits NF-kappa B and cytokine release, scavenges HOCI, and reduces viscosity of cystic fibrosis sputum.
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Ultrastructural morphology of the lung in cystic fibrosis.
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Use of rigid and flexible bronchoscopy among pediatric otolaryngologists.
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Keywords of People
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Bradford, William Dalton,
Professor of Pathology,
Pathology
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Charles, Hal Cecil,
Adjunct Associate Professor in the Department of Radiology,
Radiology
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Cohen, Seth Morris,
Professor of Head and Neck Surgery & Communication Sciences,
Head and Neck Surgery & Communication Sciences
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Jowell, Paul Simon,
Professor of Medicine,
Medicine, Gastroenterology
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Kuehn, Margarethe Joanna,
Associate Professor of Biochemistry,
Cell Biology
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Turner, David Ashley,
Consulting Professor in the Department of Pediatrics,
Pediatrics, Critical Care Medicine