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Journal J. Cyst. Fibros.

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Sleep disturbance and sleep insufficiency in primary caregivers and their children with cystic fibrosis.

Kelly C Byars, Barbara Chini, Elizabeth Hente, Raouf Amin, Thomas Boat,

BACKGROUND:Chronically ill children and their parents are at risk for sleep disorders and associated morbidity. Sleep disturbance prevalence and the relationships between parent and child sleep among children with CF are not well defined. Clarifying the presence and impact of sleep disturbances among pediatric CF patients and their parents could ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Females with cystic fibrosis have a larger decrease in sweat chloride in response to lumacaftor/ivacaftor compared to males.

B L Aalbers, R W Hofland, I Bronsveld, K M de Winter-de Groot, H G M Arets, A C de Kiviet, M M M van Oirschot-van de Ven, M A Kruijswijk, S Schotman, S Michel, C K van der Ent, H G M Heijerman,

AIM:To explore which patient-related factors influence sweat test response to CFTR modulators, as well as examining the correlation between the sweat chloride response and ppFEV1 or BMI response, using systematically collected real-life clinical data. METHODS:160 CF patients were identified who had used lumacaftor/ivacaftor for at least six months. Of these ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Global assessment of the integrated stress response in CF patient-derived airway and intestinal tissues.

Giovana B Bampi, Robert Rauscher, Sebastian Kirchner, Kathryn E Oliver, Marcel J C Bijvelds, Leonardo A Santos, Johannes Wagner, Raymond A Frizzell, Hugo R de Jonge, Eric J Sorscher, Zoya Ignatova,

BACKGROUND:Chronic inflammation is a hallmark among patients with cystic fibrosis (CF). We explored whether mutation-induced (F508del) misfolding of the cystic fibrosis transmembrane conductance regulator (CFTR), and/or secondary colonization with opportunistic pathogens, activate tissue remodeling and innate immune response drivers. METHODS:Using RNA-seq to interrogate global gene expression profiles, we analyzed stress ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Distal Intestinal Obstruction Syndrome resolved by dissolution with Coca-Cola® via colonoscopy in a cystic fibrosis patient.

Carlos Ferre-Aracil, Mariano González-Haba, Beatriz Tormo-Lanseros, Luis Giménez-Alvira, Manuel Jiménez-Garrido,

We report the case of a 37 years old woman who presented to emergency department because of a 3 days history of abdominal disconfort and distension. After physical examination and a CT scan of the abdomen she was diagnosed of Distal Intestinal Obstruction Syndrome (DIOS) in the context of her ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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COVID-19 in lung-transplanted and cystic fibrosis patients: Be careful.

Eric Farfour, Clément Picard, Laurence Beaumont, Philippe Lesprit, Marie-Christine Ballester, Felix Ackermann, Richard Galliot, Sylvie Colin de Verdiere, Charles Cerf, Marc Vasse, ,

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Can lumacaftor-ivacaftor reverse glucose-tolerance abnormalities in cystic fibrosis?

Laurence Kessler,

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Anti-inflammatory effects of lenabasum, a cannabinoid receptor type 2 agonist, on macrophages from cystic fibrosis.

Abdullah A Tarique, Tama Evron, George Zhang, Mark A Tepper, Mohammed M Morshed, Isabella S G Andersen, Nelufa Begum, Peter D Sly, Emmanuelle Fantino,

BACKGROUND:Lenabasum is an oral synthetic cannabinoid receptor type 2 agonist previously shown to reduce the production of key airway pro-inflammatory cytokines known to play a role in cystic fibrosis (CF). In a double-blinded, randomized, placebo-control phase 2 study, lenabasum lowered the rate of pulmonary exacerbation among patients with CF. The ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Average rate of lung function decline in adults with cystic fibrosis in the United Kingdom: Data from the UK CF registry.

L Caley, L Smith, H White, D G Peckham,

BACKGROUND:Rate of change in lung function is used as a measure of disease progression and a predictor of mortality in individuals with cystic fibrosis (CF). The aim of this study was to determine the national rate of decline in percent predicted Forced Expiratory Volume in 1 second (ppFEV1) in adults in ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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What it takes to implement regular longitudinal multiple breath washout tests in infants with cystic fibrosis.

Marika N Schmidt, Rikke M Sandvik, Christian Voldby, Frederik F Buchvald, Maria N Jørgensen, Per Gustafsson, Marianne Skov, Kim G Nielsen,

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Formative evaluation of a dashboard to support coproduction of healthcare services in cystic fibrosis.

Aricca D Van Citters, Alex H Gifford, Cynthia Brady, Jordan M Dunitz, Madeline Elmhirst, Jonathan Flath, Terri A Laguna, Brooke Moore, Michelle L Prickett, Maureen Riordan, Adrienne P Savant, Whitney Gore, Sarah Jian, Morgan Soper, Bruce C Marshall, Eugene C Nelson, Kathryn A Sabadosa,

BACKGROUND:Healthcare coproduction engages patients and clinicians to design and execute services, yet little is known about tools that facilitate coproduction. Our objective was to understand uptake, experiences, benefits, and limitations of a dashboard to support patient-clinician partnerships within the cystic fibrosis (CF) community. METHODS:People living with CF (PwCF) and clinicians ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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A multinational report to characterise SARS-CoV-2 infection in people with cystic fibrosis.

Rebecca Cosgriff, Susannah Ahern, Scott C Bell, Keith Brownlee, Pierre-Régis Burgel, Cass Byrnes, Harriet Corvol, Stephanie Y Cheng, Alexander Elbert, Albert Faro, Christopher H Goss, Vincent Gulmans, Bruce C Marshall, Edward McKone, Peter G Middleton, Rasa Ruseckaite, Anne L Stephenson, Siobhán B Carr,

Information is lacking on the clinical impact of the novel coronavirus, SARS-CoV-2, on people with cystic fibrosis (CF). Our aim was to characterise SARS-CoV-2 infection in people with cystic fibrosis. METHODS:Anonymised data submitted by each participating country to their National CF Registry was reported using a standardised template, then collated ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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The specialist as primary care provider in CF.

Kelsey B Haywood, Jennifer L Goralski,

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Use of a mobile application for self-management of pancreatic enzyme replacement therapy is associated with improved gastro-intestinal related quality of life in children with Cystic Fibrosis.

M Boon, J Calvo-Lerma, I Claes, T Havermans, I Asseiceira, A Bulfamante, M Garriga, E Masip, B A M van Schijndel, V Fornes, C Barreto, C Colombo, P Crespo, S Vicente, H Janssens, J Hulst, P Witters, R Nobili, L Pereira, M Ruperto, E Van der Wiel, J G Mainz, K De Boeck, C Ribes-Koninckx,

BACKGROUND:Most patients with cystic fibrosis (CF) suffer from pancreatic insufficiency (PI), leading to fat malabsorption, malnutrition, abdominal discomfort and impaired growth. Pancreatic enzyme replacement therapy (PERT) is effective, but evidence based guidelines for dose adjustment are lacking. A mobile app for self-management of PERT was developed in the context of ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Dyslipidemia is not associated with the development of glucose intolerance or diabetes in cystic fibrosis.

Johann Colomba, Rémi Rabasa-Lhoret, Anne Bonhoure, Cindy Bergeron, Valérie Boudreau, François Tremblay, Peter Senior, Kathryn Potter,

BACKGROUND:A high-fat, high-calorie diet is recommended in patients with cystic fibrosis (CF) as it improves nutritional status, respiratory health and longevity. In the general population, this diet is associated with the risk of diabetes. It is unknown whether dyslipidemic changes might contribute to the development of CF-related diabetes (CFRD). OBJECTIVE:This ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Penetrance is a critical parameter for assessing the disease liability of CFTR variants.

A Boussaroque, M-P Audrézet, C Raynal, I Sermet-Gaudelus, T Bienvenu, C Férec, A Bergougnoux, M Lopez, V Scotet, A Munck, E Girodon,

BACKGROUND:Major issues of newborn screening (NBS) for CF are the assessment of disease liability of variants and of the penetrance of clinical CF, notably in inconclusive diagnosis. The penetrance of CF is defined as the risk of a particular genotype to lead to a CF phenotype. METHODS:We aimed to get ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Endobronchial valve treatment of persistent alveolopleural fistulae in a patient with cystic fibrosis and empyema.

Kale S Bongers, Jose De Cardenas,

Persistent air leak (PAL) is a common problem after secondary pneumothorax due to cystic fibrosis (CF). These leaks, caused by either bronchopleural or alveolopleural fistula, are associated with higher morbidity and mortality [1]. Air leaks are traditionally treated with chronic chest tube drainage, chemical pleurodesis, or autologous blood patching in ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Overlooking the landscape of palliative care in cystic fibrosis.

M Chin, I D McIntosh, R Somayaji,

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Asymptomatic case of Covid-19 in an infant with cystic fibrosis.

Piercarlo Poli, Silviana Timpano, Martina Goffredo, Rita Padoan, Raffaele Badolato,

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Resting energy expenditure in cystic fibrosis patients decreases after lung transplantation, which improves applicability of prediction equations for energy requirement.

F M Hollander-Kraaijeveld, A S van Lanen, N M de Roos, E A van de Graaf, H G M Heijerman,

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Real-world evidence in cystic fibrosis modulator development: Establishing a path forward.

Amalia Magaret, Mark Warden, Noah Simon, Sonya Heltshe, Nicole Mayer-Hamblett,

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Transparency and open access in CF research.

Sanja Stanojevic, Rhonda Szczesniak,

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Caring for gender diverse youth with cystic fibrosis.

Kacie M Kidd, Gina M Sequeira, Raina V Voss, Daniel J Weiner, Bonnie W Ramsey, Raksha Jain, Traci M Kazmerski,

Gender diverse youth with cystic fibrosis have unique health needs. Providers should be aware of existing health disparities in this population as well as aspects of gender-affirming care including hormone therapy, chest binding, and use of affirming language. This communication provides an introduction to these concerns. ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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An end to casual sexism.

Andrew Bush,

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Effect of one-year lumacaftor-ivacaftor treatment on glucose tolerance abnormalities in cystic fibrosis patients.

Bastien Misgault, Eva Chatron, Quitterie Reynaud, Sandrine Touzet, Michel Abely, Laurent Melly, Stéphane Dominique, Françoise Troussier, Olivia Ronsin-Pradel, Michèle Gerardin, Julie Mankikian, Laure Cosson, Raphael Chiron, Leila Bounyar, Michel Porzio, Isabelle Durieu, Laurence Weiss, Romain Kessler, Laurence Kessler,

OBJECTIVES:To investigate the effects of 1-year lumacaftor-ivacaftor treatment on abnormalities in glucose tolerance (AGT) in Phe508del homozygous cystic fibrosis (CF) patients. METHODS:Untreated CF patients with glucose intolerance or newly diagnosed diabetes were included in a prospective, observational study. After 1-year lumacaftor-ivacaftor treatment, AGT were evaluated by using oral glucose tolerance ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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Antibiofilm and mucolytic action of nitric oxide delivered via gas or macromolecular donor using in vitro and ex vivo models.

Kaitlyn R Rouillard, David B Hill, Mark H Schoenfisch,

BACKGROUND:The combination of antibacterial and mucolytic actions makes nitric oxide (NO) an attractive dual-action cystic fibrosis (CF) therapeutic. The delivery of any therapeutic agent through pathological mucus is difficult, and the use of inhaled NO gas is inherently limited by toxicity concerns. Herein, we directly compare the ability of NO ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2020, :]

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