1,699 results match your criteria Journal of Cystic Fibrosis[Journal]


Social support is associated with fewer reported symptoms and decreased treatment burden in adults with cystic fibrosis.

J Cyst Fibros 2019 Feb 13. Epub 2019 Feb 13.

University of Colorado Denver, United States.

Background: Although social support has been linked to a variety of health outcomes in those with and without chronic illness, this construct has rarely been studied in adults with cystic fibrosis (CF), who may face a unique set of clinical and psychosocial barriers. The current study explored the relationships between social support, mental health, physical health, treatment activity, and disease-specific quality of life in a sample of adults with CF.

Methods: Participants in the study included 250 adults with CF who took part in a larger longitudinal study. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.01.013DOI Listing
February 2019
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Do patients with cystic fibrosis participating in clinical trials demonstrate placebo response? A meta-analysis.

J Cyst Fibros 2019 Feb 13. Epub 2019 Feb 13.

UMR 5558 CNRS, Equipe EMET, Université Claude Bernard Lyon 1, Lyon, France; Centre de ressources et de compétences de la mucoviscidose, Hospices Civils de Lyon, Lyon, France. Electronic address:

Background: Patients' and families' expectation that a cure for cystic fibrosis (CF) will be found is high. In other debilitating conditions, high expectation has been shown to drive a strong placebo response (PR). Therefore, our goal was to evaluate PR on objective continuous outcomes (FEV, BMI) and the CF Questionnaire Revised-Respiratory Domain (CFQR-RD) monitored during randomised clinical trials (RCTs) for CF. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.02.003DOI Listing
February 2019
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CT evaluation of hyperattenuating mucus to diagnose allergic bronchopulmonary aspergillosis in the special condition of cystic fibrosis.

J Cyst Fibros 2019 Feb 12. Epub 2019 Feb 12.

CHU de Bordeaux, Service d'Imagerie Thoracique et Cardiovasculaire, Service des Maladies Respiratoires, Service d'Exploration Fonctionnelle Respiratoire, Unité de Pneumologie pédiatrique, Service de Parasitologie-Mycologie, CIC 1401, F-33600 Pessac, France; Univ. Bordeaux, Centre de Recherche Cardio-Thoracique de Bordeaux, U1045, CIC 1401, F-33000 Bordeaux, France; Inserm, Centre de Recherche Cardio-Thoracique de Bordeaux, U1045, CIC 1401, F-33000 Bordeaux, France. Electronic address:

Background: Mucus plugging (MP), central bronchiectasis (CB), and consolidation/atelectasia (CA) are conventional CT signs to diagnose allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF). Hyperattenuating mucus (HAM) has recently been described and may improve diagnostic accuracy. The goal of our study was to compare HAM versus conventional CT signs to diagnose ABPA in CF. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.02.002DOI Listing
February 2019
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How reliable is your HbA1c test? Revisiting the use of HbA1c in cystic fibrosis-related diabetes (CFRD) screening.

J Cyst Fibros 2019 Feb 8. Epub 2019 Feb 8.

Department of Laboratory Medicine and Pathology, Faculty of Medicine and Dentistry, University of Alberta, Edmonton T6G 2R3, Canada; DynaLIFE Medical Labs, Edmonton T5J 5E2, Canada. Electronic address:

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http://dx.doi.org/10.1016/j.jcf.2019.02.001DOI Listing
February 2019
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Cystic fibrosis related diabetes is not independently associated with increased Stenotrophomonas maltophilia infection: Longitudinal data from the UK CF Registry.

J Cyst Fibros 2018 Oct 25. Epub 2018 Oct 25.

Adult Cystic Fibrosis Centre, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK. Electronic address:

Introduction: Stenotrophomonas maltophilia is common in the sputum of people with cystic fibrosis related diabetes (CFRD), raising the question as to whether this is a risk factor for its acquisition. We investigated this at a population level.

Methods: We analysed national Cystic Fibrosis Registry data 2011-2015 for 8047 people with CF > age 6 years, looking at demographics, diagnosis of CFRD, lung function and sputum microbiology; using descriptive and multivariate strategies to establish independent predictors for S. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.10.011DOI Listing
October 2018
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Incretin dysfunction and hyperglycemia in cystic fibrosis: Role of acyl-ghrelin.

J Cyst Fibros 2019 Feb 6. Epub 2019 Feb 6.

Fraternal Order of Eagles Diabetes Research Center, University of Iowa, Iowa City, IA 52242, USA; Department of Pediatrics, University of Iowa, Iowa City, IA 52242, USA; Department of Biochemistry, University of Iowa, Iowa City, IA 52242, USA. Electronic address:

Background: Insulin secretion is insufficient in cystic fibrosis (CF), even before diabetes is present, though the mechanisms involved remain unclear. Acyl-ghrelin (AG) can diminish insulin secretion and is elevated in humans with CF.

Methods: We tested the hypothesis that elevated AG contributes to reduced insulin secretion and hyperglycemia in CF ferrets. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.01.010DOI Listing
February 2019
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Authors' response: Letter to the Editor 'Comparison of lung clearance index determined by washout of N2 and SF6 in infants and preschool children with cystic fibrosis'.

J Cyst Fibros 2019 Feb 6. Epub 2019 Feb 6.

Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics, University of Heidelberg, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany; Department of Translational Pulmonology, University of Heidelberg, Im Neuenheimer Feld 156, 69120 Heidelberg, Germany; Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), Im Neuenheimer Feld 156, 69120 Heidelberg, Germany; Department of Pediatric Pulmonology, Immunology and Intensive Care Medicine, Charité-Universitätsmedizin Berlin, Augustenburger Platz 1, 13353 Berlin, Germany; Berlin Institute of Health (BIH), Anna-Louisa-Karsch-Strasse 2, 10178 Berlin, Germany. Electronic address:

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http://dx.doi.org/10.1016/j.jcf.2019.01.016DOI Listing
February 2019
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Lumacaftor-rescued F508del-CFTR has a modified bicarbonate permeability.

J Cyst Fibros 2019 Feb 6. Epub 2019 Feb 6.

CNR, Istituto di Biofisica, Genova, Italy.

Deletion of phenylalanine at position 508, F508del, the most frequent mutation among Cystic fibrosis (CF) patients, destabilizes the protein, thus causing both a folding and a trafficking defect, resulting in a dramatic reduction in expression of CFTR. In vitro treatment with lumacaftor produces an enhancement of anion transport in cells. We studied the permeability properties of the CFTR mutant F508del treated with the corrector lumacaftor, showing that the rescued protein has selectivity properties different than the wild type CFTR, showing an augmented bicarbonate permeability. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.01.012DOI Listing
February 2019
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Health economic modelling in Cystic Fibrosis: A systematic review.

J Cyst Fibros 2019 Feb 6. Epub 2019 Feb 6.

Norwich Medical School, Norwich Research Park, University of East Anglia, Norwich, Norfolk NR4 7TJ, UK.

Introduction: Cystic Fibrosis (CF) is a heritable chronic condition. Due to the genetic and progressive nature of CF, a number of interventions are available for the condition. In the United Kingdom (U. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.01.007DOI Listing
February 2019
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Methylomic correlates of autophagy activity in cystic fibrosis.

J Cyst Fibros 2019 Feb 5. Epub 2019 Feb 5.

Department of Microbial Infection and Immunity, Infectious Diseases Institute, USA; The Ohio State University, Columbus, OH 43210, USA. Electronic address:

Autophagy is a highly regulated, biological process that provides energy during periods of stress and starvation. This conserved process also acts as a defense mechanism and clears microbes from the host cell. Autophagy is impaired in Cystic Fibrosis (CF) patients and CF mice, as their cells exhibit low expression levels of essential autophagy molecules. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.01.011DOI Listing
February 2019
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Comment on "repeated hot water and steam disinfection of pari LC Plus® nebulizers alter nebulizer output".

J Cyst Fibros 2019 Feb 1. Epub 2019 Feb 1.

Northern Ireland Paediatric CF Centre, Royal Belfast Hospital for Sick Children, Northern Ireland Working Group on Nebuliser Care and Hygiene in Cystic Fibrosis, Royal Group of Hospitals, Belfast, Northern Ireland, UK.

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http://dx.doi.org/10.1016/j.jcf.2018.12.012DOI Listing
February 2019

The main mechanism associated with progression of glucose intolerance in older patients with cystic fibrosis is insulin resistance and not reduced insulin secretion capacity.

J Cyst Fibros 2019 Jan 30. Epub 2019 Jan 30.

Institut de Recherches Cliniques de Montréal, 110 avenue des Pins Ouest, Montréal H2W 1R7, Québec, Canada; Department of Nutrition, l'Université de Montréal, 2900 Boulevard Edouard-Montpetit, Montréal, QC H3T 1J4, Québec, Canada; Cystic Fibrosis Clinic, Centre Hospitalier de l'Université de Montréal (CHUM), 1051 Rue Sanguinet, Montréal, QC H2X 3E4, Québec, Canada; Department of Medicine, Université de Montréal, 2900 Boulevard Edouard-Montpetit, Montréal, QC H3T 1J4, Québec, Canada. Electronic address:

Background: Aging cystic fibrosis (CF) patients are at high risk of developing CF-related diabetes (CFRD). Decrease in insulin secretion over time is the main hypothesis to explain this increasing prevalence but mechanisms are still not well elucidated. The objective is to assess evolution of glucose tolerance and insulin secretion/sensitivity in aging CF patients. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.01.009DOI Listing
January 2019
2 Reads

Hyperbaric oxygen treatment increases killing of aggregating Pseudomonas aeruginosa isolates from cystic fibrosis patients.

J Cyst Fibros 2019 Jan 30. Epub 2019 Jan 30.

Department of Clinical Microbiology, Rigshospitalet, 2100 Copenhagen, Denmark; Costerton Biofilm Center, Department of Immunology and Microbiology, Faculty of Health Sciences University of Copenhagen, 2200 Copenhagen, Denmark. Electronic address:

Background: Pseudomonas aeruginosa is a major pathogen of the chronic lung infections in cystic fibrosis (CF) patients. These persistent bacterial infections are characterized by bacterial aggregates with biofilm-like properties and are treated with nebulized or intravenous tobramycin in combination with other antibiotics. However, the chronic infections are close to impossible to eradicate due to reasons that are far from fully understood. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.01.005DOI Listing
January 2019

Antimicrobial susceptibility testing (AST) and associated clinical outcomes in individuals with cystic fibrosis: A systematic review.

J Cyst Fibros 2019 Jan 30. Epub 2019 Jan 30.

Case Western Reserve University School of Medicine, Cleveland, OH, USA. Electronic address:

Background: Antimicrobial susceptibility testing (AST) is a cornerstone of infection management. Cystic fibrosis (CF) treatment guidelines recommend AST to select antimicrobial treatments for CF airway infection but its utility in this setting has never been objectively demonstrated.

Methods: We conducted a systematic review of primary published articles designed to address two PICO (patient, intervention, comparator, outcome) questions: 1) "For individuals with CF, is clinical response to antimicrobial treatment of bacterial airways infection predictable from AST results available at treatment initiation?" and 2) "For individuals with CF, is clinical response to antimicrobial treatment of bacterial airways infection affected by the method used to guide antimicrobial selection?" Relationships between AST results and clinical response (changes in pulmonary function, weight, signs and symptoms of respiratory tract infection, and time to next event) were assessed for each article and results were compared across articles when possible. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.01.008DOI Listing
January 2019
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Metagenome - Inferred bacterial replication rates in cystic fibrosis airways.

J Cyst Fibros 2019 Jan 23. Epub 2019 Jan 23.

Clinic for Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, D-30625 Hannover, Germany; Biomedical Research in Endstage and Obstructive Lung Disease (BREATH), German Center for Lung Research, D-30625 Hannover, Germany. Electronic address:

Bacterial replication rates were determined from metagenome sequencing of nasal lavage, throat swabs and induced sputa collected from healthy subjects and individuals with COPD or cystic fibrosis. More than 90% of peak-to-trough coverage ratios of major clones were above 1.4 indicating that the most abundant bacterial species in the microbial communities were replicating in the airways including common inhabitants such as Prevotella and Streptococcus species as well as the cystic fibrosis pathogens Staphylococcus aureus and Pseudomonas aeruginosa. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993193001
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http://dx.doi.org/10.1016/j.jcf.2019.01.003DOI Listing
January 2019
3 Reads

Establishment of a ΔF508-CF promyelocytic cell line for cystic fibrosis research and drug screening.

J Cyst Fibros 2019 Jan 7;18(1):44-53. Epub 2018 Jul 7.

Department of Microbiology, Immunology and Parasitology, Louisiana State University Health Sciences Center, New Orleans, LA 70112, USA. Electronic address:

Cystic fibrosis (CF), one of the most common genetic disorders, is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. In spite of significant improvement in patient life expectancy, the disease remains lethal and incurable. Clinically, CF lung disease claims the most morbidity and mortality, characterized by chronic bacterial infection, persistent neutrophilic inflammation, and purulent small airway obstruction. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183064
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http://dx.doi.org/10.1016/j.jcf.2018.06.007DOI Listing
January 2019
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Visceral adipose tissue is associated with poor diet quality and higher fasting glucose in adults with cystic fibrosis.

J Cyst Fibros 2019 Jan 18. Epub 2019 Jan 18.

Division of Endocrinology, Metabolism and Lipids, Department of Medicine, Emory University School of Medicine; Atlanta, GA, USA; Nutrition and Health Sciences Doctoral Program, Emory University Rollins School of Public Health, Atlanta, GA, USA; Center for Cystic Fibrosis and Airways Disease Research; Atlanta, GA, USA. Electronic address:

Background: Body fat distribution and diet quality influence clinical outcomes in general populations but are understudied in individuals with cystic fibrosis (CF). The aim of this pilot study was to assess body fat distribution and diet quality in relation to fasting glucose and lung function in adults with CF.

Methods: Subjects were 24 adults (ages 18-50) with CF and 25 age-matched controls. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183081
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http://dx.doi.org/10.1016/j.jcf.2019.01.002DOI Listing
January 2019
3 Reads

Take it personally: how personal we reach when we are so different from each other?

J Cyst Fibros 2019 Jan;18(1):6-7

SpliSense Therapeutics, Givat Ram Campus, Hebrew University, Jerusalem, Israel; Department of Genetics, The Life Sciences Institute, The Hebrew University, Jerusalem, Israel. Electronic address:

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http://dx.doi.org/10.1016/j.jcf.2018.12.009DOI Listing
January 2019
3 Reads

JCF - progress in 2018.

J Cyst Fibros 2019 Jan;18(1):1-5

Department of Medicine and Pediatrics, Medical University of South Carolina, Charleston, SC, USA.

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http://dx.doi.org/10.1016/j.jcf.2018.12.008DOI Listing
January 2019
5 Reads

Application of multiple event analysis as an alternative approach to studying pulmonary exacerbations as an outcome measure.

J Cyst Fibros 2019 Jan 11. Epub 2019 Jan 11.

Department of Biostatistics and Informatics, University of Colorado School of Public Health, USA; Department of Pediatrics, Children's Hospital Colorado, University of Colorado Anschutz Medical Center, Aurora, CO, USA.

Background: Pulmonary exacerbations (PEx) are important contributors to morbidity and mortality in cystic fibrosis (CF). Understanding risk factors for PEx is critical to improve treatment; pulmonary exacerbations also serve as an important outcome in CF clinical trials. Current risk estimates generally only evaluate time to the first PEx. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183094
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http://dx.doi.org/10.1016/j.jcf.2018.12.005DOI Listing
January 2019
10 Reads

Systemic levels of anti-PAD4 autoantibodies correlate with airway obstruction in cystic fibrosis.

J Cyst Fibros 2019 Jan 10. Epub 2019 Jan 10.

Department of Infectious Diseases, College of Veterinary Medicine, The University of Georgia, Athens, GA, USA. Electronic address:

Cystic fibrosis (CF) airway disease is characterized by the long-term presence of neutrophil granulocytes. Formation of neutrophil extracellular traps (NETs) and/or autoantibodies directed against extracellular components of NETs are possible contributors to neutrophil-mediated lung damage in CF. The goal of this study was to measure their levels in CF adults compared to healthy controls and subjects with rheumatologic diseases known to develop NET-related autoantibodies and pathologies, rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183097
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http://dx.doi.org/10.1016/j.jcf.2018.12.010DOI Listing
January 2019
3 Reads

High airborne level of Aspergillus fumigatus and presence of azole-resistant TR/L98H isolates in the home of a cystic fibrosis patient harbouring chronic colonisation with azole-resistant H285Y A. fumigatus.

J Cyst Fibros 2019 Jan 9. Epub 2019 Jan 9.

CHU Lille, Laboratoire de Parasitologie-Mycologie, F-59000 Lille, France; Univ. Lille, CNRS, Inserm, CHU Lille, Institut Pasteur de Lille, U1019 - UMR8204 - CIIL - Center for Infection and Immunity of Lille, F-59000 Lille, France. Electronic address:

Azole-resistant Aspergillus fumigatus (ARAF) has been reported in the domestic environment of patients at risk for aspergillosis. Here, we assessed the mother's and father's homes of an 18-year-old cystic fibrosis patient harbouring chronic colonisation with H285Y CYP51A azole-resistant isolate, in order to explore the link between environmental exposure and ARAF infection. In one dwelling, a very high overall contamination level was found (710-7. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993193000
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http://dx.doi.org/10.1016/j.jcf.2019.01.001DOI Listing
January 2019
4 Reads

Predictive factors for lumacaftor/ivacaftor clinical response.

J Cyst Fibros 2018 Dec 27. Epub 2018 Dec 27.

Centre Maladie Rare Mucoviscidose, Hôpital Necker-Enfants Malades, Assistance-Publique Hôpitaux de Paris, 149 rue de sèvres, 75015 Paris, France; Institut Necker-Enfants Malades, INSERM U1151, 149 rue de Sèvres, 75015 Paris, France; Université Paris Sorbonne, 75005 Paris, France. Electronic address:

Background: Ivacaftor-lumacaftor combination therapy corrects the F508 del-CFTR mutated protein which causes Cystic Fibrosis. The clinical response of the patients treated with the combination therapy is highly variable. This study aimed to determine factors involved in the individual's response to lumacaftor-ivacaftor therapy. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.12.011DOI Listing
December 2018
4 Reads

Measuring recovery in health-related quality of life during and after pulmonary exacerbations in patients with cystic fibrosis.

J Cyst Fibros 2018 Dec 23. Epub 2018 Dec 23.

Nicklaus Children's Research Institute, 3100 SW 62nd Avenue, Miami, FL 33155, USA. Electronic address:

Background: We explored the time-dependent impact of pulmonary exacerbations (PEx) on health-related quality of life (HRQoL) using Cystic Fibrosis Questionnaire-Revised (CFQ-R) data from 2 large cystic fibrosis (CF) trials.

Methods: This exploratory post-hoc analysis evaluated the impact of PEx on CFQ-R domains of functioning in 80 patients with CF (homozygous for F508del-CFTR), aged ≥14 years randomized to placebo in the TRAFFIC and TRANSPORT trials who experienced 1 PEx.

Results: Scores on the CFQ-R were significantly lower within 1 week of PEx start in 8 out of 12 domains (Respiratory Symptoms, Physical Functioning, Emotional Functioning, Health Perceptions, Role Functioning, Social Functioning, Eating, and Vitality). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183094
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http://dx.doi.org/10.1016/j.jcf.2018.12.004DOI Listing
December 2018
7 Reads

A multicenter evaluation of sweat chloride concentration and variation in infants with cystic fibrosis.

J Cyst Fibros 2018 Dec 21. Epub 2018 Dec 21.

Department of Pediatrics, University of Colorado Denver School of Medicine, Breathing Institute, Children's Hospital of Colorado, Aurora, CO, United States.

Fifty-nineCF infants' sweat chloride concentrations were analyzed to answer the questions: What is the biological and analytical variation in sweat chloride concentrations collected from the 32 infants homozygous for the F508 deletion? Do sweat chloride concentrations change in the first year of life beyond the variance previously established for adults with similar CFTR mutations? The biological and analytical variation of the infants' sweat chloride concentration was similar to that seen in adult CF patients. While there was a statistically significant difference between sweat chloride concentration in early (89.8 mmol/L) and late (95. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.12.006DOI Listing
December 2018
1 Read

Differences in the lower airway microbiota of infants with and without cystic fibrosis.

J Cyst Fibros 2018 Dec 20. Epub 2018 Dec 20.

Department of Respiratory and Sleep Medicine, Royal Children's Hospital, Victoria, Australia; Respiratory Diseases Group, Murdoch Childrens Research Institute, Victoria, Australia; Department of Paediatrics, University of Melbourne, Victoria, Australia.

Background: Cystic fibrosis (CF) lung disease commences in infancy, and understanding the role of the microbiota in disease pathogenesis is critical. This study examined and compared the lower airway microbiota of infants with and without CF and its relationship to airway inflammation in the first months of life.

Methods: Infants newly-diagnosed with CF were recruited into a single-centre study in Melbourne, Australia from 1992 to 2001. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.12.003DOI Listing
December 2018
1 Read

Isogenic cell models of cystic fibrosis-causing variants in natively expressing pulmonary epithelial cells.

J Cyst Fibros 2018 Dec 15. Epub 2018 Dec 15.

Cystic Fibrosis Foundation, CFFT Lab, 44 Hartwell Ave., Lexington, MA 02421, USA. Electronic address:

Background: Assessment of approved drugs and developmental drug candidates for rare cystic fibrosis (CF)-causing variants of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) requires abundant material from relevant models.

Methods: Isogenic cell lines harboring CFTR variants in the native genomic context were created through the development and utilization of a footprint-less, CRISPR/Cas9 gene editing pipeline in 16HBE14o- immortalized bronchial epithelial cells.

Results: Isogenic, homozygous cell lines for three CFTR variants (F508del and the two most common CF-causing nonsense variants, G542X and W1282X) were established and characterized. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.12.001DOI Listing
December 2018
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Cirrhosis associated with decreased survival and a 10-year lower median age at death of cystic fibrosis patients in the Netherlands.

J Cyst Fibros 2018 Dec 14. Epub 2018 Dec 14.

The University Medical Center Groningen, Dept. of Pediatrics, University of Groningen, Groningen, the Netherlands, Hanzeplein 1, 9713 GZ Groningen, The Netherlands. Electronic address:

Background: Up to 10% of patients with Cystic Fibrosis develop cirrhotic CF-related liver disease with portal hypertension: CF cirrhosis (CFC). In a nationwide study, we aimed to determine the role of CFC on survival in the Netherlands between 1 and 1-2009 and1-1-2015.

Methods: We identified all CFC patients in the Netherlands, based on ultrasonographic liver nodularity and portal hypertension. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.11.009DOI Listing
December 2018
2 Reads

Letter to the editor: Cystic fibrosis in the Middle East: An awareness analysis.

Authors:
Ahmad M Hider

J Cyst Fibros 2018 Dec 12. Epub 2018 Dec 12.

Department of Medicine, University of Cambridge, School of Clinical Medicine, Cambridge CB2 0SP, United Kingdom. Electronic address:

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http://dx.doi.org/10.1016/j.jcf.2018.12.002DOI Listing
December 2018
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The increasing challenge of genetic counseling for cystic fibrosis.

J Cyst Fibros 2018 Dec 7. Epub 2018 Dec 7.

Children's Hospital Los Angeles, Pediatric Pulmonology Division, Department of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033, United States. Electronic address:

Genetic counseling for cystic fibrosis (CF) is challenged by intricate molecular mechanisms, complex phenotypes, and psychosocial needs. CFTR variant interpretation has become critical; this manuscript examines variant nomenclature and classes, as well as opportunities and challenges posed by genetic technologies and genotype-directed therapies. With post-graduate training in medical genetics and counseling, genetic counselors educate patients and families, facilitate testing and interpretation, and help integrate genetic information into diagnosis and treatment. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.11.014DOI Listing
December 2018

Depletion of BAFF cytokine exacerbates infection in Pseudomonas aeruginosa infected mice.

J Cyst Fibros 2018 Dec 7. Epub 2018 Dec 7.

Department of Human Genetics, McGill University, Montreal, Quebec, Canada; Program in Infectious Diseases and Immunity in Global Health, McGill University Health Center, Montreal, Quebec, Canada. Electronic address:

Background: Cystic fibrosis (CF) is a genetic disease characterized by chronic inflammation of the lungs that is ineffective at clearing pathogens. B-cell activating factor (BAFF), a cytokine involved in the development of B-cells, is known to be elevated in CF patients with subclinical infections. We postulate that the elevated BAFF levels in CF patients might be triggered by Pseudomonas aeruginosa infection and it might play a protective role in the regulation of lung responses to infection. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.11.015DOI Listing
December 2018
1 Read

Antibody response against Mycobacterium avium complex in cystic fibrosis patients measured by a novel IgG ELISA test.

J Cyst Fibros 2018 Nov 29. Epub 2018 Nov 29.

Department of Clinical Microbiology, Rigshospitalet, Copenhagen, Denmark; Institute of Immunology and Medical Microbiology, University of Copenhagen, Denmark.

Background: Early signs of Mycobacterium avium complex pulmonary disease can be missed in patients with cystic fibrosis due to subclinical infection or delays in mycobacterial culture. The aim of this study was to determine the diagnostic accuracy of a novel enzyme linked immunosorbent assay for immunoglobulin G against Mycobacterium avium complex, which could help stratify patients according to risk.

Methods: A retrospective cross sectional analysis of serum samples from the Copenhagen Cystic Fibrosis Center was performed. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.11.011DOI Listing
November 2018
1 Read

Colocolonic intussusception in an adult cystic fibrosis patient.

J Cyst Fibros 2018 Nov 28. Epub 2018 Nov 28.

Gregory Fleming James Cystic Fibrosis Research Center Birmingham, AL, USA; The Department of Medicine, University of Alabama at Birmingham Birmingham, AL, USA. Electronic address:

Purpose: To raise awareness of colocolonic intussusception as a gastrointestinal complication of CF mimicking distal intestinal obstruction syndrome (DIOS) and discuss risk of recurrence.

Case Summary: A 33-year-old Caucasian male with cystic fibrosis presented with an acute abdomen diagnosed via imaging as colocolonic intussusception. He was managed with fluid replacement therapy and polyethylene glycol. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.11.013DOI Listing
November 2018
2 Reads

Non-invasive ventilation and clinical outcomes in cystic fibrosis: Findings from the UK CF registry.

J Cyst Fibros 2018 Nov 28. Epub 2018 Nov 28.

National Heart and Lung Institute, Imperial College London, United Kingdom.

Background: Non-invasive ventilation (NIV) for respiratory failure and airway clearance is an established intervention in cystic fibrosis (CF), but its therapeutic benefit on lung function and survival remains under-investigated.

Methods: Using data from the UK CF Registry between 2007 and 2015, we explored the patterns of NIV use, and assessed changes in mean percent predicted FEV (ppFEV) prior to and after NIV use, and the survival of patients on NIV.

Results: Among 11,079 patients, 1107 had at least one record of NIV treatment. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.11.006DOI Listing
November 2018
1 Read

Beyond the expected: Identifying broad research priorities of researchers and the cystic fibrosis community.

J Cyst Fibros 2018 Nov 28. Epub 2018 Nov 28.

Cystic Fibrosis Foundation, Bethesda, MD, United States; Jacobs School of Medicine and Biomedical Sciences, Buffalo, NY, United States.

The Cystic Fibrosis Foundation (CFF) supports research programs aimed at improving care and building a successful drug development pipeline. To ensure its research agenda meets the needs of the community it serves, the CFF sought community input into clinical research prioritization for topics not well-known as already being addressed by CFF-funded research. In 2018, clinical researchers, adults with CF, and family members were surveyed about a broad range of research topics that are perceived to receive less attention. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.11.010DOI Listing
November 2018
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Prevalence and diversity of filamentous fungi in the airways of cystic fibrosis patients - A Dutch, multicentre study.

J Cyst Fibros 2018 Dec 1. Epub 2018 Dec 1.

Center of Expertise in Mycology Radboudumc/CWZ, Nijmegen, The Netherlands; Department of Medical Microbiology and Infectious Diseases, Canisius Wilhelmina Hospital (CWZ), Nijmegen, The Netherlands.

Background: Progressive lung injury in Cystic Fibrosis (CF) patients can lead to chronic colonization with bacteria and fungi. Fungal colonization is obtained from the environment which necessitates locally performed epidemiology studies. We prospectively analyzed respiratory samples of CF patients during a 3-year period, using a uniform fungal culture protocol, focusing on filamentous fungi and azole resistance in Aspergillus fumigatus. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.11.012DOI Listing
December 2018
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Controlled attenuation parameter: A measure of hepatic steatosis in patients with cystic fibrosis.

J Cyst Fibros 2018 Nov 30. Epub 2018 Nov 30.

Division of Gastroenterology, Hepatology, and Nutrition, Boston Children's Hospital, Boston, MA, United States. Electronic address:

Background: Hepatic steatosis is a common manifestation of CF-related liver disease(CFLD). Controlled attenuation parameter(CAP) measurement during transient elastography(TE) semiquantifies liver steatosis. We examined the relationship between CAP and CFLD severity, clinical factors and liver stiffness measurements(LSM). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183093
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http://dx.doi.org/10.1016/j.jcf.2018.11.004DOI Listing
November 2018
10 Reads

The lung and gut microbiome: what has to be taken into consideration for cystic fibrosis?

J Cyst Fibros 2019 Jan 25;18(1):13-21. Epub 2018 Nov 25.

Halo Research Group, School of Pharmacy, Queen's University Belfast, Belfast, UK.

The 15th European Cystic Fibrosis Society (ECFS) Basic Science pre-conference Symposium focused on the topic of the microbiome, asking the question "The lung and gut microbiome: what has to be considered for cystic fibrosis (CF)?" This review gives an overview of the main points raised during the symposium, which dealt with the technical considerations, pathophysiology and clinical implications of the microbiome in CF. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.11.003DOI Listing
January 2019

Pre-transplant factors associated with mortality after lung transplantation in cystic fibrosis: A systematic review and meta-analysis.

J Cyst Fibros 2018 Oct 25. Epub 2018 Oct 25.

Division of Respirology, Adult Cystic Fibrosis Centre, St. Michael's Hospital, Toronto, ON, Canada.

Background: Mortality risk stratification is essential in lung transplantation (LTx) to allow listing, prioritization and mitigating strategies. In cystic fibrosis (CF) patients, predictors of post-LTx mortality are not established.

Methods: For this systematic review and meta-analysis, seven databases were searched until January 3, 2018 to identify predictors of post-LTx mortality in CF. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.10.013DOI Listing
October 2018

Urinary metabolomics reveals unique metabolic signatures in infants with cystic fibrosis.

J Cyst Fibros 2018 Nov 23. Epub 2018 Nov 23.

Cystic Fibrosis Foundation, Bethesda, MD, USA; Department of Pediatrics, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY, USA.

Background: Biologic pathways and metabolic mechanisms underpinning early systemic disease in cystic fibrosis (CF) are poorly understood. The Baby Observational and Nutrition Study (BONUS) was a prospective multi-center study of infants with CF with a primary aim to examine the current state of nutrition in the first year of life. Its secondary aim was to prospectively explore concurrent nutritional, metabolic, respiratory, infectious, and inflammatory characteristics associated with early CF anthropometric measurements. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183091
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http://dx.doi.org/10.1016/j.jcf.2018.10.016DOI Listing
November 2018
4 Reads

Dental and periodontal manifestations in patients with cystic fibrosis - A systematic review.

J Cyst Fibros 2018 Nov 22. Epub 2018 Nov 22.

Department of Pulmonology, Allergology and Respiratory Oncology, Poznan University of Medical Sciences, Szamarzewskiego 82/84, Poznań 60-569, Poland.

Background: The oral cavity is an important reservoir of microorganisms and can be a potential source of infection of the respiratory tract in CF patients. The literature on the oral health does not explicitly determine dental problems in these patients. The aim of the study was to systematically review the studies relating to oral status in CF patients. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.11.007DOI Listing
November 2018
3 Reads

Cystic fibrosis is associated with an increased risk of Barrett's esophagus.

J Cyst Fibros 2018 Nov 22. Epub 2018 Nov 22.

Division of Digestive & Liver Diseases, Columbia University Medical Center, New York, NY, USA.

Background: Cystic fibrosis (CF) patients have increased risks of gastrointestinal cancers, including esophageal adenocarcinoma. Gastroesophageal reflux disease (GERD) is highly prevalent in CF and manifests at early ages. CF patients may be at increased risk for long-term sequelae of chronic GERD, including Barrett's esophagus (BE). Read More

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http://dx.doi.org/10.1016/j.jcf.2018.11.005DOI Listing
November 2018
1 Read

Antisense oligonucleotide eluforsen improves CFTR function in F508del cystic fibrosis.

J Cyst Fibros 2018 Nov 19. Epub 2018 Nov 19.

University of Alabama at Birmingham, 2000 6(th) Avenue S., Birmingham, AL 35233, USA.

Background: Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. In this study we assessed the effect of antisense oligonucleotide eluforsen on CFTR biological activity measured by Nasal Potential Difference (NPD) in patients with the most common mutation, F508del-CFTR.

Methods: This multi-centre, exploratory, open-label study recruited adults with CF homozygous or compound heterozygous for the F508del-CFTR mutation. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.10.015DOI Listing
November 2018
12 Reads

Pseudomonas aeruginosa colonization causes PD-L1 overexpression on monocytes, impairing the adaptive immune response in patients with cystic fibrosis.

J Cyst Fibros 2018 Nov 12. Epub 2018 Nov 12.

Innate Immunity Group, IdiPAZ, La Paz University Hospital, Madrid, Spain; Tumor Immunology Lab, IdiPAZ, La Paz University Hospital, Madrid, Spain; Center for Biomedical Research Network, CIBEres, Spain. Electronic address:

Background: Cystic fibrosis (CF) is an endotoxin tolerance (ET)-related disease. Given that increased PD-L1 has been reported in ET, its expression and physiological effects on cystic fibrosis monocytes should be studied.

Methods: We analyzed the phenotype and ex vivo response of immune system cells in 32 patients with CF, 19 of them colonized by Pseudomonas aeruginosa. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.11.002DOI Listing
November 2018
8 Reads

Comparison of lung clearance index determined by washout of N and SF in infants and preschool children with cystic fibrosis.

J Cyst Fibros 2018 Nov 9. Epub 2018 Nov 9.

Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics, University of Heidelberg, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany; Department of Translational Pulmonology, University of Heidelberg, Im Neuenheimer Feld 156, 69120 Heidelberg, Germany; Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), Im Neuenheimer Feld 156, 69120 Heidelberg, Germany; Department of Pediatric Pulmonology, Immunology and Intensive Care Medicine, Charité-Universitätsmedizin Berlin, Augustenburger Platz 1, 13353 Berlin, Germany; Berlin Institute of Health (BIH), Anna-Louisa-Karsch-Strasse 2, 10178 Berlin, Germany. Electronic address:

Background: Multiple-breath washout (MBW) has been shown to detect early impairment of lung function in children with cystic fibrosis (CF). Nitrogen (N) or sulfur hexafluoride (SF) can be used as tracer gas for MBW. Recent data indicated higher lung clearance index (LCI) values measured with N-MBW than concurrent SF-MBW in older children and adults, however, a comparison in infants and younger children, as well as to other outcome measures of CF lung disease is pending. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183092
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http://dx.doi.org/10.1016/j.jcf.2018.11.001DOI Listing
November 2018
9 Reads

Glycosaminoglycans are differentially involved in bacterial binding to healthy and cystic fibrosis lung cells.

J Cyst Fibros 2018 Nov 8. Epub 2018 Nov 8.

University Institute Fernandez-Vega (IUFV), University of Oviedo and Eye Research Foundation (FIO), Oviedo, Spain; Instituto de Investigación Sanitaria del Principado de Asturias (IISPA), Oviedo, Spain; Department of Functional Biology, University of Oviedo, Oviedo, Spain. Electronic address:

Background: Glycosaminoglycans (GAGs) are essential in many infections, including recurrent bacterial respiratory infections, the main cause of mortality in cystic fibrosis (CF) patients.

Methods: Using a cellular model of healthy and CF lung epithelium, a comparative transcriptomic study of GAG encoding genes was performed using qRT-PCR, and their differential involvement in the adhesion of bacterial pathogens analyzed by enzymatic degradation and binding competition experiments.

Results: Various alterations in gene expression in CF cells were found which affect GAG structures and seem to influence bacterial adherence to lung epithelium cells. Read More

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http://dx.doi.org/10.1016/j.jcf.2018.10.017DOI Listing
November 2018

Validation of the French 3-year prognostic score using the Canadian Cystic Fibrosis registry.

J Cyst Fibros 2018 Nov 2. Epub 2018 Nov 2.

Adult CF Centre, Department of Respirology, St. Michael's Hospital, Toronto, Ontario M5B 1W8, Canada; Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto M5T 3M6, Ontario, Canada; Keenan Research Center, Li Ka Shing Knowledge Institute of St. Michael's Hospital, Toronto M5B 1T8, Ontario, Canada. Electronic address:

Studies of large CF populations using registry data are important to identify people at high risk for death. Nkam et al. published a prognostic score developed on French CF registry data to predict death or lung transplantation (LT) over a 3-year period in the adult CF population. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183091
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http://dx.doi.org/10.1016/j.jcf.2018.10.014DOI Listing
November 2018
6 Reads

Functional characterization and phenotypic spectrum of three recurrent disease-causing deep intronic variants of the CFTR gene.

J Cyst Fibros 2018 Oct 30. Epub 2018 Oct 30.

Université de Montpellier, Laboratoire de Génétique de Maladies Rares, EA7402 Montpellier, France.

Background: The CFTR genotype remains incomplete in 1% of Cystic Fibrosis (CF) cases, because only one or no disease-causing variants is detected after extended analysis. This fraction is probably higher in CFTR-Related Disorders (CFTR-RD). Deep-intronic CFTR variants are putative candidates to fill this gap. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183089
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http://dx.doi.org/10.1016/j.jcf.2018.10.012DOI Listing
October 2018
9 Reads

Higher Interleukin-7 serum concentrations in patients with cystic fibrosis correlate with impaired lung function.

J Cyst Fibros 2019 Jan 31;18(1):71-77. Epub 2018 Oct 31.

Department of General Pediatrics, Neonatology, and Pediatric Cardiology, University Children's Hospital, Medical Faculty, Heinrich Heine University, 40225 Duesseldorf, Germany. Electronic address:

Background: Patients with cystic fibrosis (CF) are highly susceptible to infection and colonization of pulmonary epithelia. Repeated and chronic infections may affect disease course and efficacy of host immune protection. Higher Interleukin (IL)-7 serum levels, indicating impaired T-cell response to IL-7, have been described for chronic viral and mycobacterial infections. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183089
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http://dx.doi.org/10.1016/j.jcf.2018.09.008DOI Listing
January 2019
9 Reads

Failure to conceive in women with CF is associated with pancreatic insufficiency and advancing age.

J Cyst Fibros 2018 Oct 23. Epub 2018 Oct 23.

Cystic Fibrosis Center, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.

Objective: The causes of subfertility in women with CF though multifactorial are not well described. Our aim in this study was to determine the prevalence and factors associated with female subfertility among women with CF.

Methods: A retrospective multinational study from 11 CF centers in 5 countries (Israel, France, Spain, Italy, UK) including women with CF was undertaken. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993183086
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http://dx.doi.org/10.1016/j.jcf.2018.10.009DOI Listing
October 2018
20 Reads