1,725 results match your criteria Journal of Cystic Fibrosis[Journal]


Cystic fibrosis screen positive inconclusive diagnosis (CFSPID): Experience in Tuscany, Italy.

J Cyst Fibros 2019 Apr 17. Epub 2019 Apr 17.

Cystic Fibrosis Centre, Department of Paediatric Medicine, Anna Meyer Children's University Hospital, Florence, Italy.

Objective: The implementation of cystic fibrosis (CF) newborn screening (NBS) has led to identification of infants with a positive NBS test but inconclusive diagnosis classified as "CF screen positive, inconclusive diagnosis" (CFSPID). We retrospectively evaluated the prevalence and clinical outcome of CFSPID infants diagnosed by 2 NBS algorithms in the period from 2011 to 2016 in the Tuscany region of Italy.

Methods: In 2011-2016, we assessed the diagnostic impact of DNA analysis on the NBS 4-tier algorithm [immunoreactive trypsin (IRT) - meconium lactase - IRT2 - sweat chloride (SC)]. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.04.002DOI Listing
April 2019
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Addressing lung transplant with adults with cystic fibrosis: A qualitative analysis of patients' perspectives and experiences.

J Cyst Fibros 2019 Apr 16. Epub 2019 Apr 16.

Department of Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine, University of Washington, Seattle, WA, USA.

Referral for lung transplantation is a complex process that typically begins with a discussion in cystic fibrosis (CF) clinic. We performed a secondary analysis of interviews conducted at the University of Washington CF Clinic as part of a study of unmet palliative care needs, June 2015 - January 2016, among adults with moderate-to-severe CF-related lung disease. Content analysis methods were used to identify themes related to discussion of lung transplant in CF clinic. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.04.007DOI Listing

Patient engagement to prioritise CF research: Inclusive or selective?

J Cyst Fibros 2019 Apr 11. Epub 2019 Apr 11.

Nottingham Evidence Based Child Health Group, University of Nottingham, Division of Child Health, Obstetrics & Gynaecology, United Kingdom. Electronic address:

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http://dx.doi.org/10.1016/j.jcf.2019.04.006DOI Listing

Using registries for research in CF. How can we be sure about the outputs?

J Cyst Fibros 2019 Apr 11. Epub 2019 Apr 11.

Department of Pediatrics, University of Toronto and Research Institute, Hospital for Sick Children, Toronto, Canada. Electronic address:

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http://dx.doi.org/10.1016/j.jcf.2019.04.003DOI Listing

Cystic fibrosis dyslipidaemia: A cross-sectional study.

J Cyst Fibros 2019 Apr 9. Epub 2019 Apr 9.

Poznan University of Medical Sciences, Department of Pediatric Gastroenterology and Metabolic Diseases, Poznan, Poland. Electronic address:

Background: The interest in cystic fibrosis (CF) dyslipidaemia as a potential risk factor for cardiovascular disease is increasing with patients' survival. This study aimed to investigate CF dyslipidaemia, its clinical correlates and links to oxidized low-density lipoprotein (oxLDL), adiponectin, and apolipoprotein E (APOE).

Methods: This cross-sectional study assessed clinical characteristics of CF, as well as the serum lipid profile, oxLDL, adiponectin, and APOE. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.04.001DOI Listing
April 2019
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Assessment of endothelial function is reproducible in patients with cystic fibrosis.

J Cyst Fibros 2019 Apr 4. Epub 2019 Apr 4.

Department of Population Health Sciences, Augusta University, Augusta, GA, United States of America; Georgia Prevention Institute, Department of Population Health Sciences, Augusta University, Augusta, GA, United States of America; Sport and Exercise Science Research Institute, University of Ulster, Jordanstown, Northern Ireland, UK. Electronic address:

Background: New treatments have improved life-expectancy in patients with cystic fibrosis (CF); however, cardiovascular health remains an area of concern in this population. Flow-mediated dilation (FMD), a non-invasive assessment of vascular endothelial function that predicts future cardiovascular disease and events, is attenuated in patients with CF compared to controls. The reproducibility of FMD in CF; however, has yet to be evaluated. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.03.011DOI Listing
April 2019
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The impact of cystic fibrosis-related diabetes on health-related quality of life.

J Cyst Fibros 2019 Mar 29. Epub 2019 Mar 29.

Division of Respiratory Medicine, Department of Medicine, University of British Columbia, Vancouver, Canada; Centre for Heart Lung Innovation, St Paul's Hospital and the University of British Columbia, Vancouver, Canada. Electronic address:

Cystic fibrosis-related diabetes (CFRD) is a well-known comorbidity among the CF population. To investigate whether CFRD impacts health-related quality of life (HRQoL), domain scores from the Cystic Fibrosis Questionnaire-Revised for adolescents and adults over 14 years old (CFQ-R 14+) were compared between CF individuals with CFRD on insulin, CFRD not on insulin, impaired glucose tolerance, and normal blood glucose tolerance. The median score for the Treatment Burden domain was significantly worse for individuals with CFRD on insulin (p < 0. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.03.007DOI Listing
March 2019
4 Reads

Markers of bone turnover are reduced in patients with CF related diabetes; the role of glucose.

J Cyst Fibros 2019 Mar 27. Epub 2019 Mar 27.

Copenhagen Cystic Fibrosis Center, Department of Infectious Diseases, Rigshospitalet, Copenhagen University Hospital, Blegdamsvej 9, DK2100 Copenhagen, Denmark.

Background: Cystic fibrosis(CF) related diabetes(CFRD) and osteoporosis are prevalent in adult patients with CF. We aimed to evaluate if CFRD and markers of glucose metabolism and inflammation are associated with bone turnover in CF.

Methods: Cross sectional study at the adult section at the Copenhagen CF Center from January-October 2017. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.01.004DOI Listing
March 2019
2 Reads

Aligning patients' needs and research priorities towards a comprehensive CF research program.

J Cyst Fibros 2019 Mar 26. Epub 2019 Mar 26.

University Medical Center Utrecht (UMCU), the Netherlands.

The Dutch CF Foundation started to focus on scientific research thirteen years ago. The patient organization defined the patients perspective and unmet needs bottom-up, and through a structured process. The patients research priorities were matched with the research priorities of Dutch basic scientists and clinicians. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.03.008DOI Listing
March 2019
2 Reads

Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines.

J Cyst Fibros 2019 Mar 27. Epub 2019 Mar 27.

Division of Pulmonary, Allergy, Critical Care Medicine and Sleep, Dept of Medicine, University of Minnesota, Minneapolis, MN, USA. Electronic address:

Objective: Provide recommendations to the cystic fibrosis (CF) community to facilitate timely referral for lung transplantation for individuals with CF.

Methods: The CF Foundation organized a multidisciplinary committee to develop CF Lung Transplant Referral Consensus Guidelines. Three workgroups were formed: timing for transplant referral; modifiable barriers to transplant; and transition to transplant care. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.03.002DOI Listing
March 2019
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Physical activity and associations with clinical outcome measures in adults with cystic fibrosis; a systematic review.

J Cyst Fibros 2019 Mar 26. Epub 2019 Mar 26.

Research Institute for Sport and Exercise Sciences, Liverpool John Moores University, Liverpool L3 3AF, United Kingdom.

Background: Physical activity (PA) is important in the management of Cystic Fibrosis (CF) and is associated with a number of beneficial effects. PA assessment is not commonplace or consistent in clinical practice, therefore understanding of PA in adults with CF remains limited. The purpose of this review was to evaluate PA levels in this population and compare PA to global recommendations and non-CF peers. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.03.003DOI Listing
March 2019
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A two-center analysis of hyperpolarized Xe lung MRI in stable pediatric cystic fibrosis: Potential as a biomarker for multi-site trials.

J Cyst Fibros 2019 Mar 25. Epub 2019 Mar 25.

Translational Medicine Program, The Hospital for Sick Children, Toronto, ON, Canada; Department of Medical Biophysics, University of Toronto, Toronto, ON, Canada.. Electronic address:

Background: The ventilation defect percent (VDP), measured from hyperpolarized (HP) Xe magnetic resonance imaging (MRI), is sensitive to functional changes in cystic fibrosis (CF) lung disease. The purpose of this study was to measure and compare VDP from HP Xe MRI acquired at two institutions in stable pediatric CF subjects with preserved lung function.

Methods: This retrospective analysis included 26 participants from two institutions (18 CF, 8 healthy, age range 10-17). Read More

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http://dx.doi.org/10.1016/j.jcf.2019.03.005DOI Listing
March 2019
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Assessment of stability and fluctuations of cultured lower airway bacterial communities in people with cystic fibrosis.

J Cyst Fibros 2019 Mar 21. Epub 2019 Mar 21.

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

Background: Routine clinical culture detects a subset of the cystic fibrosis (CF) airways microbiota based on culture-independent (molecular) methods. This study aimed to determine how extended sputum culture of viable bacteria changes over time in relation to clinical status and predicts exacerbations.

Methods: Sputa from patients at a baseline stable and up to three subsequent time-points were analysed by extended-quantitative culture; aerobe/anaerobe densities, ecological indexes and community structure were assessed together with clinical outcomes. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.02.012DOI Listing
March 2019
1 Read

Cystic fibrosis in the modern therapeutic era: Give the shower a thought!

J Cyst Fibros 2019 Mar 20. Epub 2019 Mar 20.

Pediatric Pulmonology and cystic fibrosis center, La Timone-Enfants University Hospital, Marseille, France; Aix-Marseille Univ, IRD, APHM, Mephi, IHU Méditerranée Infection, Marseille, France. Electronic address:

Fifty-two meshes of e-flow rapid® were characterized for tobramycin delivery with a laser diffractometer after 6 months of home use by cystic fibrosis patients treated with various nebulized drugs. Three meshes were out of order and 30 considered to be defective for tobramycin delivery. The use of the specific mesh cleaning shower system permitted 14 defective meshes to be in the expected range of nebulized volume. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.03.004DOI Listing
March 2019
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Effects of Lumacaftor/Ivacaftor on physical activity and exercise tolerance in three adults with cystic fibrosis.

J Cyst Fibros 2019 Mar 14. Epub 2019 Mar 14.

Department of Public Health and Infectious Diseases, Adult Cystic Fibrosis Center, Sapienza University of Rome, 00185 Rome, Italy; Eleonora Lorrillard-Spencer Cenci Foundation, 00185 Rome, Italy. Electronic address:

The combination of the corrector lumacaftor with the potentiator ivacaftor has been approved for treatment of cystic fibrosis (CF) patients homozygous for the Phe508del CFTR mutation. There are no reports detailing the effect of lumacaftor-ivacaftor on physical activity (PA) and exercise tolerance. We performed incremental cardiopulmonary exercise testing (CPET) and we assessed PA pre- and post 2 years initiation of lumacaftor-ivacaftor in three CF adults. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.03.001DOI Listing
March 2019
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The effects of cycled inhaled aztreonam on the cystic fibrosis (CF) lung microbiome.

J Cyst Fibros 2019 Mar 8. Epub 2019 Mar 8.

Department of Microbiology, Immunology and Infectious Diseases, University of Calgary, Calgary, AB, Canada; Department of Medicine, University of Calgary, Calgary, AB, Canada. Electronic address:

Background: To improve clinical outcomes, cystic fibrosis (CF) patients with chronic Pseudomonas aeruginosa infections are prescribed inhaled anti-pseudomonal antibiotics. Although, a diverse microbial community exists within CF airways, little is known about how the CF microbiota influences patient outcomes. We hypothesized that organisms within the CF microbiota are affected by inhaled-antibiotics and baseline microbiome may be used to predict therapeutic response. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.02.010DOI Listing
March 2019
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Investigating transmission of Mycobacterium abscessus amongst children in an Australian cystic fibrosis centre.

J Cyst Fibros 2019 Mar 7. Epub 2019 Mar 7.

Department of Microbiology, Royal Children's Hospital, 50 Flemington Rd, Parkville, Victoria, Australia; Department of Infection Prevention and Control, Royal Children's Hospital, 50 Flemington Rd, Parkville, Victoria, Australia; Department of Paediatrics, The University of Melbourne, Victoria, Australia. Electronic address:

Background: Mycobacterium abscessus is an emerging pathogen in cystic fibrosis (CF) lung disease. Hospital transmission of M. abscessus has been described. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.02.011DOI Listing
March 2019
2 Reads

Comment on Comparison of lung clearance index determined by washout of N2 and SF6 in infants and preschool children with cystic fibrosis.

J Cyst Fibros 2019 Mar 1. Epub 2019 Mar 1.

Division of Allergy, Immunology and Pulmonology, Department of Pediatrics, Childrens Hospital of Pittsburgh of UPMC, Pittsburgh, PA, USA.

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http://dx.doi.org/10.1016/j.jcf.2018.12.007DOI Listing
March 2019
3 Reads

Abnormal preschool Lung Clearance Index (LCI) reflects clinical status and predicts lower spirometry later in childhood in cystic fibrosis.

J Cyst Fibros 2019 Feb 28. Epub 2019 Feb 28.

Department of Respiratory Medicine, The Children's Hospital at Westmead (CHW), Sydney, Australia; Discipline of Paediatrics and Child Health, Sydney Medical School, University of Sydney, Australia. Electronic address:

Background: Clinical and prognostic value of preschool Multiple Breath Washout (MBW) remains unclear.

Methods: Initial MBW results (Exhalyzer® D, EcoMedics AG) in preschool Cystic Fibrosis (CF) subjects (age 2-6 years) at a time of clinical stability were compared to (1) concurrent clinical status measures and (2) later spirometry outcomes. Abnormal Lung Clearance Index (LCI) was defined using published reference data (ULN for LCI 8. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.02.007DOI Listing
February 2019
1 Read

Early respiratory viral infections in infants with cystic fibrosis.

J Cyst Fibros 2019 Feb 27. Epub 2019 Feb 27.

Department of Pediatrics, The University of North Carolina at Chapel Hill School of Medicine, Chapel Hill, NC, United States.

Background: Viral infections contribute to morbidity in cystic fibrosis (CF), but the impact of respiratory viruses on the development of airway disease is poorly understood.

Methods: Infants with CF identified by newborn screening were enrolled prior to 4 months of age to participate in a prospective observational study at 4 centers. Clinical data were collected at clinic visits and weekly phone calls. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.02.004DOI Listing
February 2019
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Associating antimicrobial susceptibility testing with clinical outcomes in cystic fibrosis: More rigor and less frequency?

J Cyst Fibros 2019 Mar 26;18(2):159-160. Epub 2019 Feb 26.

Division of Pulmonary and Sleep Medicine, University of Washington, Seattle Children's Hospital, United States.

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http://dx.doi.org/10.1016/j.jcf.2019.02.008DOI Listing
March 2019
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Decreased mRNA and protein stability of W1282X limits response to modulator therapy.

J Cyst Fibros 2019 Feb 22. Epub 2019 Feb 22.

McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, United States. Electronic address:

Background: Cell-based studies have shown that W1282X generates a truncated protein that can be functionally augmented by modulators. However, modulator treatment of primary cells from individuals who carry two copies of W1282X generates no functional CFTR. To understand the lack of response to modulators, we investigated the effect of W1282X on CFTR RNA transcript levels. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15691993193002
Publisher Site
http://dx.doi.org/10.1016/j.jcf.2019.02.009DOI Listing
February 2019
7 Reads

Biofabrication of personalised anatomical models and tools for the clinic.

J Cyst Fibros 2019 Mar 21;18(2):161-162. Epub 2019 Feb 21.

Institute of Health and Biomedical Innovation, Queensland University of Technology (QUT), 60 Musk Avenue, Kelvin Grove, Brisbane, QLD 4059, Australia.

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http://dx.doi.org/10.1016/j.jcf.2019.02.005DOI Listing
March 2019
1 Read

Cystic fibrosis: We see fungus among us, but should we care?

J Cyst Fibros 2019 Mar 21;18(2):163-164. Epub 2019 Feb 21.

Johns Hopkins University, Baltimore, MD, USA.

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http://dx.doi.org/10.1016/j.jcf.2019.02.006DOI Listing
March 2019
3 Reads

Interprofessional provider educational needs and preferences regarding the provision of sexual and reproductive health care in cystic fibrosis.

J Cyst Fibros 2019 Feb 19. Epub 2019 Feb 19.

Division of Respiratory Diseases, Department of Pediatrics, Boston Children's Hospital, Boston, MA, United States of America.

Background: Sexual and reproductive health (SRH) is an important disease-specific concern for adolescent and young adult (AYA) women that is not consistently addressed in cystic fibrosis (CF) centers. This study identifies educational needs and preferences of interprofessional CF providers regarding SRH in AYA women with CF.

Methods: Interprofessional CF providers participated in an anonymous survey regarding general and CF-specific SRH knowledge and skills, factors for optimizing SRH care, and preferred approaches for SRH training. Read More

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http://dx.doi.org/10.1016/j.jcf.2019.01.015DOI Listing
February 2019
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Repeated hot water and steam disinfection of pari LC Plus® nebulizers alters nebulizer output.

J Cyst Fibros 2019 Mar 18;18(2):e17-e18. Epub 2019 Feb 18.

Associate Medical Director, Hospital Epidemiology Yale New Haven Children's Hospital, Department of Infectious Disease, Yale New Haven Children's Hospital, USA.

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http://dx.doi.org/10.1016/j.jcf.2019.01.014DOI Listing
March 2019
2 Reads

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
3 Reads

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
2 Reads

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 Mar 8;18(2):e14-e15. 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
March 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 2019 Mar 25;18(2):294-298. 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
March 2019
2 Reads

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
1 Read

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
2 Reads

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
3 Reads

Comment on "repeated hot water and steam disinfection of pari LC Plus® nebulizers alter nebulizer output".

J Cyst Fibros 2019 Mar 2;18(2):e16. Epub 2019 Feb 2.

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

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
5 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
1 Read

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

J Cyst Fibros 2019 Mar 30;18(2):236-243. 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
March 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
9 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
5 Reads

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
7 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
4 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
9 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
14 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
9 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
9 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
7 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
12 Reads