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Preclinical atherosclerosis in cystic fibrosis: Two distinct presentations are related to pancreatic status
Cross CE Reverri EJ Morrissey BM. Joining the crowd: cystic fibrosis and cardiovascular disease risk factors.Chest. 2013; ...
Satisfaction and effectiveness of opioid pain management among adults with cystic fibrosis: A mixed methods study
Highlights•High satisfaction and effectiveness with opioid pain management plans were reported•Effectiveness and satisfact...
Immunoglobulin GM and KM allotypes are associated with antibody responses to Pseudomonas aeruginosa antigens in chronically infected cystic fibrosis patients
Background Chronic infection with Pseudomonas aeruginosa (P. aeruginosa) is a leading cause of death in patie...
Antisense oligonucleotide-based drug development for Cystic Fibrosis patients carrying the 3849+10 kb C-to-T splicing mutation
1. IntroductionCystic fibrosis (CF) is a life-shortening multi-organ genetic disease affecting ~ 90,000 individuals worldw...
Entry of cystic fibrosis transmembrane conductance potentiator ivacaftor into the developing brain and lung
Background The potential effects of ivacaftor during pregnancy and breastfeeding on the offspring ar...
The association between body composition, leptin levels and glucose dysregulation in youth with cystic fibrosis
Cystic fibrosis–related diabetes: current trends in prevalence, incidence, and mortality.Diab Care. 32Cystic-Fibrosis Rela...
Breast development in a 7 year old girl with CF treated with ivacaftor: An indication for personalized dosing?
Highlights•Breast development was observed as a side effect of treatment with ivacaftor.•Plasma concentration of ivacaftor...
Association between insurance variability and early lung function in children with cystic fibrosis
Background Lower socioeconomic status (SES) has consistently been associated with poorer outcomes in...
Review of Gastrointestinal Motility in Cystic Fibrosis
De Lisle R.C. Borowitz D. The cystic fibrosis intestine.Cold Spring Harb Perspect Med. 3 ()Cystic Fibrosis Disease Modifie...
VIP reduction in the pancreas of F508del homozygous CF mice and early signs of Cystic Fibrosis Related Diabetes (CFRD)
1. IntroductionVasoactive intestinal peptide (VIP), which was discovered in the 1970s by Said and Mutt, is a 28-residue po...
Risk factors for cystic fibrosis arthropathy: Data from the German cystic fibrosis registry
1. IntroductionDuring the last decades, median age of survival of cystic fibrosis (CF) patients has increased significantl...
Actigraphic and patient and family reported sleep outcomes in children and youth with cystic fibrosis: A systematic review
Identifying patient priorities for psychosocial support in an adult cystic fibrosis clinic.Pediatric Pulmonology. 54: 421I...
Epigenome editing of the CFTR-locus for treatment of cystic fibrosis
Use of a high-throughput phenotypic screening strategy to identify amplifiers, a novel pharmacological class of small mole...
Overweight and obesity in adults with cystic fibrosis: An Italian multicenter cohort study
Background Over the last decades aggressive interventions have been successful to improve nutritional ...
Effect of lumacaftor-ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis: Results from the PROSPECT MCC sub-study
Wainwright CE Elborn JS Ramsey BW Marigowda G Huang X Cipolli M et al.Lumacaftor-ivacaftor in patients with cystic fibrosi...
Respiratory symptoms do not reflect functional impairment in early CF lung disease
1 Swiss Cystic Fibrosis Infant Lung Development (SCILD) cohort, current study group: Juerg Barben, MD, St. Gallen; Sylvain...
Sex differences in treatment patterns in cystic fibrosis pulmonary exacerbations
Background Females with cystic fibrosis (CF) have been shown to have worse pulmonary exacerbation (P...
HOMA indices as screening tests for cystic fibrosis-related diabetes
Background We assessed the diagnostic performances of homeostasis model assessment indices (HOMA) of...
Clinical impact of levofloxacin inhalation solution in cystic fibrosis patients in a real-world setting
Background Levofloxacin inhalation solution is the most recently approved inhaled antibiotic in Euro...
Comparison of Cas9 and Cas12a CRISPR editing methods to correct the W1282X-CFTR mutation
Background W1282X-CFTR variant (c.3846G>A) is the second most common nonsense cystic fibrosis (CF...
Speaking of pandemics...
We are in the midst of a persistent pandemic that threatens the health of the population we care for, particularly i...
Phenotypic characteristics of incident and chronic MRSA isolates in cystic fibrosis
Dasenbrook E.C. Checkley W. Merlo C.A. Konstan M.W. Lechtzin N. Boyle M.P. Association between respiratory tract methicill...
Microbial interaction: Prevotella spp. reduce P. aeruginosa induced inflammation in cystic fibrosis bronchial epithelial cells
Background In Cystic Fibrosis (CF) airways, the dehydrated, thick mucus promotes the establishment o...
Cystic fibrosis-related diabetes (CFRD) and cognitive function in adults with cystic fibrosis
Moran A.M. Dunitz J. Nathan B. Saeed A. Holme B. Thomas W. Cystic fibrosis-related diabetes: current trends in prevalence,...
Non-invasive ventilation is associated with long-term improvements in lung function and gas exchange in cystic fibrosis adults with hypercapnic respiratory failure
Manchester Adult Cystic Fibrosis Centre, Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, M23...
Cardiovascular complications in cystic fibrosis: A review of the literature
Cystic fibrosis.Lancet. 388: 2519-2531Immune responses in cystic fibrosis: are they intrinsically defective?.Am J Respir C...
Long-term amikacin liposome inhalation suspension in cystic fibrosis patients with chronic P. aeruginosa infection
Cystic fibrosis.Lancet. 2016; 388: 2519-2531Clancy J.P. Cotton C.U. Donaldson S.H. Solomon G.M. VanDevanter D.R. Boyle M.P...
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