Cystic Fibrosis

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  Subject Areas on Research

  • 5' exon replacement and repair by spliceosome-mediated RNA trans-splicing. 
  • A cystic fibrosis transmembrane conductance regulator splice variant with partial penetrance associated with variable cystic fibrosis presentations. 
  • A matched comparison of adjustment in children with cystic fibrosis and psychiatrically referred and nonreferred children. 
  • A review of rectal biopsy. 
  • A revised airway epithelial hierarchy includes CFTR-expressing ionocytes. 
  • AAV exploits subcellular stress associated with inflammation, endoplasmic reticulum expansion, and misfolded proteins in models of cystic fibrosis. 
  • ACT with CF: A telehealth and in-person feasibility study to address anxiety and depressive symptoms among people with cystic fibrosis. 
  • Abnormal bone turnover in cystic fibrosis adults. 
  • Acrodermatitis enteropathica-like eruption and food allergy. 
  • Acute renal failure in cystic fibrosis: association with inhaled tobramycin therapy. 
  • Adverse alterations in bone metabolism are associated with lung infection in adults with cystic fibrosis. 
  • Age of Pseudomonas aeruginosa acquisition and subsequent severity of cystic fibrosis lung disease. 
  • An approach for treating the hepatobiliary disease of cystic fibrosis by somatic gene transfer. 
  • Annual decline in lung function in adults with sickle cell disease is similar to that observed in adults with cystic fibrosis. 
  • Association of Transition Readiness to Intentional Self-Regulation and Hopeful Future Expectations in Youth With Illness. 
  • Atrial fibrillation after pulmonary transplant. 
  • Barriers to adherence to cystic fibrosis infection control guidelines. 
  • Basal-like cells constitute the proliferating cell population in cystic fibrosis airways. 
  • Bcl-2 suppresses sarcoplasmic/endoplasmic reticulum Ca2+-ATPase expression in cystic fibrosis airways: role in oxidant-mediated cell death. 
  • Bilateral sequential lung and simultaneous pancreas transplant: a new approach for the recipient with cystic fibrosis. 
  • Biochemical and biophysical identification of cystic fibrosis transmembrane conductance regulator chloride channels as components of endocytic clathrin-coated vesicles. 
  • Bronchiectasis: Mechanisms and Imaging Clues of Associated Common and Uncommon Diseases. 
  • Burden of influenza-related hospitalizations among children with sickle cell disease. 
  • CFTR as a cAMP-dependent regulator of sodium channels. 
  • CFTR haplotype analysis reveals genetic heterogeneity in the etiology of congenital bilateral aplasia of the vas deferens. 
  • CFTR: development of high- affinity antibodies and localization in sweat gland. 
  • CHEER National Study of Chronic Rhinosinusitis Practice Patterns: Disease Comorbidities and Factors Associated with Surgery. 
  • Case 6. Abnormal facies and mental retardation in a patient with cystic fibrosis of the pancreas. 
  • Cellular and viral splicing factors can modify the splicing pattern of CFTR transcripts carrying splicing mutations. 
  • Change over a 12-month period in the psychological adjustment of children and adolescents with cystic fibrosis. 
  • Characterization of the cystic fibrosis transmembrane conductance regulator in a colonocyte cell line. 
  • Children with Chronic Lung Disease: Facilitating Smoking Cessation for their Caregivers. 
  • Combined lung-liver-pancreas transplantation in a recipient with cystic fibrosis. 
  • Comparison of US Federal and Foundation Funding of Research for Sickle Cell Disease and Cystic Fibrosis and Factors Associated With Research Productivity. 
  • Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. 
  • Coping and quality of life in patients awaiting lung transplantation. 
  • Correlation between sinus and lung cultures in lung transplant patients with cystic fibrosis. 
  • Cystic fibrosis and the National Institutes of Health consensus statement: are obstetrician-gynecologists ready to comply? 
  • Cystic fibrosis gene mutations and pancreatitis risk: relation to epithelial ion transport and trypsin inhibitor gene mutations. 
  • Cystic fibrosis in Jews: frequency and mutation distribution. 
  • Cystic fibrosis in a black woman. 
  • Cystic fibrosis in the pancreas: recent advances provide new insights. 
  • Cystic fibrosis involving the cervix, mimicking a well-differentiated adenocarcinoma: a case report. 
  • Cystic fibrosis mutations and genetic predisposition to idiopathic chronic pancreatitis. 
  • Cystic fibrosis pulmonary guidelines: pulmonary complications: hemoptysis and pneumothorax. 
  • Cystic fibrosis: combined hyperpolarized 3He-enhanced and conventional proton MR imaging in the lung--preliminary observations. 
  • Cystic fibrosis: usefulness of thoracic CT in the examination of patients before lung transplantation. 
  • Decreased Antibiotic Utilization After Sinus Surgery in Cystic Fibrosis Patients With Lung Transplantation. 
  • Defective innate immunity and hyperinflammation in newborn cystic fibrosis transmembrane conductance regulator-knockout ferret lungs. 
  • Desensitization to pancreatic enzyme intolerance in a child with cystic fibrosis. 
  • Developing Breakthrough Drugs for Heart Failure: Lessons Learned From the Cystic Fibrosis Experience. 
  • Diminished and erratic absorption of ergocalciferol in adult cystic fibrosis patients. 
  • Direct-from-blood RNA sequencing identifies the cause of post-bronchoscopy fever. 
  • Disruption of iron homeostasis in the lungs of transplant patients. 
  • Dynamic perfluorinated gas MRI reveals abnormal ventilation despite normal FEV1 in cystic fibrosis. 
  • Dysregulation of proteoglycan production by intrahepatic biliary epithelial cells bearing defective (delta-f508) cystic fibrosis transmembrane conductance regulator. 
  • EUR-1008 pancreatic enzyme replacement is safe and effective in patients with cystic fibrosis and pancreatic insufficiency. 
  • Effect of single vs bilateral lung transplantation on plasma surfactant protein D levels in idiopathic pulmonary fibrosis. 
  • Efficacy of alendronate in adults with cystic fibrosis with low bone density. 
  • Efficacy of pamidronate for osteoporosis in patients with cystic fibrosis following lung transplantation. 
  • Electron microprobe analysis of human labial gland secretory granules in cystic fibrosis. 
  • Endoscopic Sinus Surgery for Cystic Fibrosis: Variables Influencing Sinonasal and Pulmonary Outcomes. 
  • Epigenome editing of the CFTR-locus for treatment of cystic fibrosis. 
  • Evaluation of Antimicrobial Susceptibility Testing Methods for Burkholderia cenocepacia and Burkholderia multivorans Isolates from Cystic Fibrosis Patients. 
  • 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. 
  • Expression of the cystic fibrosis gene in adult human lung. 
  • Failure to thrive, diarrhea, cough, and oral candidiasis in a three-month-old boy. 
  • Fatal respiratory infection due to ST308 VIM-1-producing Pseudomonas aeruginosa in a lung transplant recipient: case report and review of the literature. 
  • Gastroesophageal reflux as a reversible cause of allograft dysfunction after lung transplantation. 
  • Gastrointestinal case of the day. Cystic fibrosis with colonic intussusception. 
  • Glucose tolerance, insulin secretion, and insulin sensitivity in children and adolescents with cystic fibrosis and no prior history of diabetes. 
  • Head and neck manifestations of cystic fibrosis and ciliary dyskinesia. 
  • Hepatic parenchymal cells in cystic fibrosis: ultrastructural evidence for abnormal intracellular transport. 
  • Homozygous sickle cell disease and cystic fibrosis in an adolescent. 
  • Hsp 70/Hsp 90 organizing protein as a nitrosylation target in cystic fibrosis therapy. 
  • Human cystic fibrosis transmembrane conductance regulator directed to respiratory epithelial cells of transgenic mice. 
  • Hypoxic Gene Expression of Donor Bronchi Linked to Airway Complications after Lung Transplantation. 
  • Idiopathic pancreatitis related to CFTR: complex inheritance and identification of a modifier gene. 
  • Illness specific patterns of psychological adjustment and cognitive adaptational processes in children with cystic fibrosis and sickle cell disease. 
  • Immunohistologic quantification of Pseudomonas aeruginosa in the tracheobronchial tree from patients with cystic fibrosis. 
  • Impact of Gene Patents and Licensing Practices on Access to Genetic Testing for Cystic Fibrosis 
  • Incorporating patient and caregiver feedback into lung transplant referral guidelines for individuals with cystic fibrosis-Preliminary findings from a novel paradigm. 
  • Increased risk of idiopathic chronic pancreatitis in cystic fibrosis carriers. 
  • Infections in patients with cystic fibrosis following lung transplantation. 
  • Inflammatory cell distribution within and along asthmatic airways. 
  • Injection laryngoplasty in children with cystic fibrosis and abnormal swallow. 
  • Inspissation of pancreatic zymogen material in cystic fibrosis. 
  • Internal consistency of DSM-III diagnoses using the symptom scales of the Child Assessment Schedule. 
  • 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. 
  • Iron accumulates in the lavage and explanted lungs of cystic fibrosis patients. 
  • Kaposi sarcoma after bilateral lung transplantation. 
  • Klotho Inhibits Interleukin-8 Secretion from Cystic Fibrosis Airway Epithelia. 
  • Localization of the cystic fibrosis transmembrane conductance regulator in human bile duct epithelial cells. 
  • Localization of the cystic fibrosis transmembrane conductance regulator in pancreas. 
  • 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. 
  • Loss of cftr function leads to pancreatic destruction in larval zebrafish. 
  • Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines. 
  • Lung transplantation for cystic fibrosis patients with Burkholderia cepacia complex. Survival linked to genomovar type. 
  • Major and massive hemoptysis: reassessment of conservative management. 
  • Mapping and correcting hyperpolarized magnetization decay with radial keyhole imaging. 
  • Mapping strategies for multiple linked markers. 
  • Medical and obstetric complications among pregnant women with cystic fibrosis. 
  • Mild cystic fibrosis in a consanguineous family. 
  • Mild cystic fibrosis linked to chromosome 7q22 markers with an uncommon haplotype. 
  • Missense mutation (G480C) in the CFTR gene associated with protein mislocalization but normal chloride channel activity. 
  • Molecular basis of defective anion transport in L cells expressing recombinant forms of CFTR. 
  • 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. 
  • Mutations of the cystic fibrosis gene and pancreatitis. 
  • Mycobacterium abscessus chest wall and pulmonary infection in a cystic fibrosis lung transplant recipient. 
  • Nasally inhaled dornase alfa in the postoperative management of chronic sinusitis due to cystic fibrosis. 
  • Novel human bronchial epithelial cell lines for cystic fibrosis research. 
  • Novel techniques for MR imaging of pulmonary airspaces. 
  • Opportunities to Improve Utilization of Palliative Care Among Adults With Cystic Fibrosis: A Systematic Review. 
  • Parenteral administration of oral medications in lung transplant recipients: An underrecognized problem. 
  • Partial correction of endogenous DeltaF508 CFTR in human cystic fibrosis airway epithelia by spliceosome-mediated RNA trans-splicing. 
  • Pharmacological modulation of the AKT/microRNA-199a-5p/CAV1 pathway ameliorates cystic fibrosis lung hyper-inflammation. 
  • Phosphorylation of the cystic fibrosis transmembrane conductance regulator. 
  • Physiorack: an integrated MRI safe/conditional, gas delivery, respiratory gating, and subject monitoring solution for structural and functional assessments of pulmonary function. 
  • Preferences and Stated Adherence for Antibiotic Treatment of Cystic Fibrosis Pseudomonas Infections. 
  • Protein phosphorylation responses in normal and cystic fibrosis airway epithelial cell lines. 
  • Pseudomonas aeruginosa Leucine Aminopeptidase Influences Early Biofilm Composition and Structure via Vesicle-Associated Antibiofilm Activity. 
  • Pseudomonas aeruginosa vesicles associate with and are internalized by human lung epithelial cells. 
  • Pseudomonas colonization in cystic fibrosis. A study of 160 patients. 
  • Pseudomonas invasion of type I pneumocytes is dependent on the expression and phosphorylation of caveolin-2. 
  • Psychological adjustment of children with cystic fibrosis: the role of child cognitive processes and maternal adjustment. 
  • Pulmonary embolization of microcrystalline cellulose in a lung transplant recipient. 
  • Purification of outer membrane vesicles from Pseudomonas aeruginosa and their activation of an IL-8 response. 
  • Reduced CFTR function and the pathobiology of idiopathic pancreatitis. 
  • Regulation of membrane chloride currents in rat bile duct epithelial cells. 
  • Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis. 
  • Repair of CFTR mRNA by spliceosome-mediated RNA trans-splicing. 
  • 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. 
  • Respiratory syncytial virus infection enhances Pseudomonas aeruginosa biofilm growth through dysregulation of nutritional immunity. 
  • 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? 
  • Roles of mucus adhesion and cohesion in cough clearance. 
  • SARS-CoV-2 Reverse Genetics Reveals a Variable Infection Gradient in the Respiratory Tract. 
  • Scanning electron microscopic study of the airways in normal children and in patients with cystic fibrosis and other lung diseases. 
  • Screening of CFTR mutations in an isolated population: identification of carriers and patients. 
  • Secretory Cells Dominate Airway CFTR Expression and Function in Human Airway Superficial Epithelia. 
  • Serum IgD concentrations in normal infants, children, and adults and in patients with elevated IgE. 
  • Short-term calcitriol administration improves calcium homeostasis in adults with cystic fibrosis. 
  • Significance of blood stream infection after lung transplantation: analysis in 176 consecutive patients. 
  • Simultaneous bilateral lung and pancreas transplantation in recipient with cystic fibrosis. 
  • Six-year molecular analysis of Burkholderia cepacia complex isolates among cystic fibrosis patients at a referral center for lung transplantation. 
  • 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). 
  • Splenorenal shunt with underlying cirrhosis of the liver. 
  • Stability and change in the psychological adjustment of mothers of children and adolescents with cystic fibrosis and sickle cell disease. 
  • Stress, coping, and family functioning in the psychological adjustment of mothers of children and adolescents with cystic fibrosis. 
  • Sublingual tacrolimus for immunosuppression in lung transplantation: a potentially important therapeutic option in cystic fibrosis. 
  • Submucosal glands are the predominant site of CFTR expression in the human bronchus. 
  • Survey of cystic fibrosis transmembrane conductance regulator genotypes in primary sclerosing cholangitis. 
  • Survival Benefit of Lung Transplantation in the Modern Era of Lung Allocation. 
  • Survival after lung transplantation of cystic fibrosis patients infected with Burkholderia cepacia complex. 
  • Survival of lung transplant patients with cystic fibrosis harboring panresistant bacteria other than Burkholderia cepacia, compared with patients harboring sensitive bacteria. 
  • The impact of cystic fibrosis and PSTI/SPINK1 gene mutations on susceptibility to chronic pancreatitis. 
  • The molecular basis of disease variability among cystic fibrosis patients carrying the 3849+10 kb C-->T mutation. 
  • The role of biomedical and psychosocial processes in the intellectual and academic functioning of children and adolescents with cystic fibrosis. 
  • The role of cystic fibrosis gene mutations in determining susceptibility to chronic pancreatitis. 
  • The wait for the waitlist: The next challenge in the lung allocation system. 
  • To Splice or Not to Splice, That Is the Treatment. 
  • Trends in the clinical characteristics of the U.S. cystic fibrosis patient population from 1995 to 2005. 
  • Tyloxapol inhibits NF-kappa B and cytokine release, scavenges HOCI, and reduces viscosity of cystic fibrosis sputum. 
  • Ultrastructural morphology of the lung in cystic fibrosis. 
  • Use of rigid and flexible bronchoscopy among pediatric otolaryngologists. 
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  Keywords of People

  • Bradford, William Dalton, Professor of Pathology, Pathology
  • Charles, Hal Cecil, Associate Professor of Radiology, Radiology
  • Cohen, Seth Morris, Professor of Head and Neck Surgery & Communication Sciences, Head and Neck Surgery & Communication Sciences
  • Jowell, Paul Simon, Professor of Medicine, Medicine, Gastroenterology
  • Kuehn, Margarethe Joanna, Associate Professor of Biochemistry, Molecular Genetics and Microbiology
  • Turner, David Ashley, Consulting Professor in the Department of Pediatrics, Pediatrics, Critical Care Medicine