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Cystic Fibrosis

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https://www.readbyqxmd.com/read/28222269/restoring-cftr-function-reduces-airway-bacteria-and-inflammation-in-people-with-cystic-fibrosis-and-chronic-lung-infections
#1
Katherine B Hisert, Sonya L Heltshe, Christopher Pope, Peter Jorth, Xia Wu, Rachael M Edwards, Matthew Radey, Frank J Accurso, Daniel J Wolter, Gordon Cooke, Ryan J Adam, Suzanne Carter, Brenda Grogan, Jan L Launspach, Seamas C Donnelly, Charles Gallagher, James E Bruce, David Stoltz, Michael J Welsh, Lucas R Hoffman, Edward F McKone, Pradeep K Singh
RATIONALE: Previous work indicates that ivacaftor improves CFTR activity and lung function in people with cystic fibrosis (CF) and G551D-CFTR mutations, but does not reduce density of bacteria or markers of inflammation in the airway. These findings raise the possibility that infection and inflammation may progress independently of CFTR activity once CF lung disease is established. OBJECTIVES: To better understand the relationship between CFTR activity, airway microbiology and inflammation, and lung function in subjects with CF and chronic airway infections...
February 21, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28222031/a-retrospective-analysis-of-longitudinal-changes-in-bone-mineral-content-in-cystic-fibrosis
#2
Adela Chirita-Emandi, Sheila Shepherd, Andreas Kyriakou, Jane D McNeilly, Carol Dryden, Donna Corrigan, Anne Devenny, Syed Faisal Ahmed
BACKGROUND: We aimed to describe the longitudinal changes in bone mineral content and influencing factors, in children with cystic fibrosis (CF). METHODS: One hundred children (50 females) had dual X-ray absorptiometry (DXA) performed. Of these, 48 and 24 children had two to three scans, respectively over 10 years of follow-up. DXA data were expressed as lumbar spine bone mineral content standard deviation score (LSBMCSDS) adjusted for age, gender, ethnicity and bone area...
February 21, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28221736/individual-pharmacokinetic-variation-leads-to-underdosing-of-ciprofloxacin-in-some-cystic-fibrosis-patients
#3
A N Ø Schultz, N Høiby, X C Nielsen, T Pressler, K Dalhoff, M Duno, A Buchard, H K Johansen, H Wang, C S Dalbøge
Ciprofloxacin (CIP) is frequently used when treating cystic fibrose (CF) patients with intermittent Pseudomonas aeruginosa (P. aeruginosa) lung colonization. However, approximately 20% of the patients progress to chronic infection despite early intervention. The aim of this study, was to investigate the pharmacokinetics of CIP, to evaluate if CYP3A4-related metabolism is involved and to find the optimal dose needed to eradicate intermittently colonizing bacteria in the lungs of CF patients. Methods An open-label, prospective pharmacokinetic study was performed...
March 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28220034/exhaled-and-nasal-nitric-oxide-in-relation-to-lung-function-blood-cell-counts-and-disease-characteristics-in-cystic-fibrosis
#4
Christina Krantz, Christer Janson, Annika Hollsing, Kjell Alving, Andrei Malinovschi
BACKGROUND: Patients with CF have similar or lower exhaled nitric oxide (FeNO) and lower nasal nitric oxide (nNO) levels than controls. There are divergent results on alveolar NO (CalvNO) concentrations in relation to CF. There are inconsistent results on correlation between different nitric oxide parameters and lung function and inflammation in CF. AIM: To compare FeNO, CalvNO and nNO levels between subjects with CF, asthma and healthy controls and to study whether these parameters are related to lung function, blood cell counts or clinical characteristics in CF patients...
February 21, 2017: Journal of Breath Research
https://www.readbyqxmd.com/read/28219060/steady-state-therapy-with-azithromycin-or-low-dose-prednisolone-in-paediatric-cystic-fibrosis-patients-inflammatory-markers-and-disease-progression
#5
Galina Shmarina, Alexander Pukhalsky, Lucine Avakian, Sergey Semykin, Daria Pukhalskaya, Vladimir Alioshkin
BACKGROUND: Anti-inflammatory therapy is a logical approach to slowing the inevitable lung function deterioration in cystic fibrosis (CF) patients. This study's aim was to evaluate inflammatory markers and disease progression in paediatric CF patients chronically treated with azithromycin or low-dose prednisolone. METHODS: The study included 204 patients with CF and 100 healthy controls; 102 CF patients were treated with basic therapy only (without anti-inflammatory treatment; WAT), and 102 individuals received basic therapy along with azithromycin (n = 59) or low-dose prednisolone (n = 43)...
February 21, 2017: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/28218802/non-invasive-ventilation-for-cystic-fibrosis
#6
REVIEW
Fidelma Moran, Judy M Bradley, Amanda J Piper
BACKGROUND: Non-invasive ventilation may be a means to temporarily reverse or slow the progression of respiratory failure in cystic fibrosis by providing ventilatory support and avoiding tracheal intubation. Using non-invasive ventilation, in the appropriate situation or individuals, can improve lung mechanics through increasing airflow and gas exchange and decreasing the work of breathing. Non-invasive ventilation thus acts as an external respiratory muscle. This is an update of a previously published review...
February 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28218655/taste-receptors-mediate-sinonasal-immunity-and-respiratory-disease
#7
REVIEW
Jennifer E Douglas, Noam A Cohen
The bitter taste receptor T2R38 has been shown to play a role in the pathogenesis of chronic rhinosinusitis (CRS), where the receptor functions to enhance upper respiratory innate immunity through a triad of beneficial immune responses. Individuals with a functional version of T2R38 are tasters for the bitter compound phenylthiocarbamide (PTC) and exhibit an anti-microbial response in the upper airway to certain invading pathogens, while those individuals with a non-functional version of the receptor are PTC non-tasters and lack this beneficial response...
February 17, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28218486/determinants-and-outcomes-of-upfront-surgery-versus-medical-therapy-for-chronic-rhinosinusitis-in-cystic-fibrosis
#8
Noel Ayoub, Andrew Thamboo, Al-Rahim Habib, Jayakar V Nayak, Peter H Hwang
BACKGROUND: The indications for surgical management of chronic rhinosinusitis (CRS) in patients with cystic fibrosis (CF) are poorly defined. In this study we compare outcomes of medical versus surgical treatment and examine trends associated with the transition from medical to surgical therapy in CF patients. METHODS: One hundred thirty-six patients with CF referred to a tertiary rhinology practice were retrospectively divided into 3 cohorts: Medical, Upfront Surgery, or Crossover, if they converted from medical to surgical management...
February 20, 2017: International Forum of Allergy & Rhinology
https://www.readbyqxmd.com/read/28215711/biomarkers-for-cystic-fibrosis-drug-development
#9
REVIEW
Marianne S Muhlebach, J P Clancy, Sonya L Heltshe, Assem Ziady, Tom Kelley, Frank Accurso, Joseph Pilewski, Nicole Mayer-Hamblett, Elizabeth Joseloff, Scott D Sagel
PURPOSE: To provide a review of the status of biomarkers in cystic fibrosis drug development, including regulatory definitions and considerations, a summary of biomarkers in current use with supportive data, current gaps, and future needs. METHODS: Biomarkers are considered across several areas of CF drug development, including cystic fibrosis transmembrane conductance regulator modulation, infection, and inflammation. RESULTS: Sweat chloride, nasal potential difference, and intestinal current measurements have been standardized and examined in the context of multicenter trials to quantify CFTR function...
November 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28213960/association-of-rhinovirus-with-exacerbations-in-young-children-affected-by-cystic-fibrosis-preliminary-data
#10
Sacha Stelzer-Braid, Nancy Liu, Michael Doumit, Russell D'Cunha, Yvonne Belessis, Adam Jaffe, William D Rawlinson
Rhinovirus (RV) is a common respiratory viral infection linked to worsening of chronic respiratory diseases including cystic fibrosis (CF) and asthma. RV was tested by RT-PCR in samples (n = 465) collected from the upper (nasal swab, oropharyngeal suction and sputum) and lower (bronchoalveolar washings) respiratory tract of 110 children with CF. Air samples (n = 52) collected from the operating theatres and outpatient clinics were tested for RV. RV was found in 43% of children <5 years suffering an exacerbation, and 12% of older children (5-17 years)...
February 18, 2017: Journal of Medical Virology
https://www.readbyqxmd.com/read/28213469/insulin-signaling-via-the-pi3k-akt-pathway-regulates-airway-glucose-uptake-and-barrier-function-in-a-cftr-dependent-manner
#11
Samuel A Molina, Hannah K Moriarty, Danny T Infield, Barry R Imhoff, Rachel J Vance, Agnes H Kim, Jason M Hansen, William R Hunt, Michael Koval, Nael A McCarty
Cystic fibrosis-related diabetes (CFRD) is the most common co-morbidity associated with cystic fibrosis (CF) and correlates with increased rates of lung function decline. Since glucose is a nutrient present in the airways of patients with bacterial airway infections and since insulin controls glucose metabolism, the effect of insulin on CF airway epithelia was investigated to determine the role of insulin receptors and glucose transport in regulating glucose availability in the airway. The response to insulin by human airway epithelial cells was characterized by qPCR, immunoblot, immunofluorescence, and glucose uptake assays...
February 17, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28211475/biofilm-formation-of-the-black-yeast-like-fungus-exophiala-dermatitidis-and-its-susceptibility-to-antiinfective-agents
#12
Lisa Kirchhoff, Maike Olsowski, Katrin Zilmans, Silke Dittmer, Gerhard Haase, Ludwig Sedlacek, Eike Steinmann, Jan Buer, Peter-Michael Rath, Joerg Steinmann
Various fungi have the ability to colonize surfaces and to form biofilms. Fungal biofilm-associated infections are frequently refractory to targeted treatment because of resistance to antifungal drugs. One fungus that frequently colonises the respiratory tract of cystic fibrosis (CF) patients is the opportunistic black yeast-like fungus Exophiala dermatitidis. We investigated the biofilm-forming ability of E. dermatitidis and its susceptibility to various antiinfective agents and natural compounds. We tested 58 E...
February 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28210295/global-impact-of-bronchiectasis-and-cystic-fibrosis
#13
REVIEW
Margarida Redondo, Holly Keyt, Raja Dhar, James D Chalmers
EDUCATIONAL AIMS: To recognise the clinical and radiological presentation of the spectrum of diseases associated with bronchiectasis.To understand variation in the aetiology, microbiology and burden of bronchiectasis and cystic fibrosis across different global healthcare systems. : Bronchiectasis is the term used to refer to dilatation of the bronchi that is usually permanent and is associated with a clinical syndrome of cough, sputum production and recurrent respiratory infections...
September 2016: Breathe
https://www.readbyqxmd.com/read/28209466/pharmacokinetics-and-safety-of-cavosonstat-n91115-in-healthy-and-cystic-fibrosis-adults-homozygous-for-f508del-cftr
#14
Scott H Donaldson, George M Solomon, Pamela L Zeitlin, Patrick A Flume, Alicia Casey, Karen McCoy, Edith T Zemanick, Arun Mandagere, Janice M Troha, Steven A Shoemaker, James F Chmiel, Jennifer L Taylor-Cousar
BACKGROUND: Cavosonstat (N91115), an orally bioavailable inhibitor of S-nitrosoglutathione reductase, promotes cystic fibrosis transmembrane conductance regulator (CFTR) maturation and plasma membrane stability, with a mechanism of action complementary to CFTR correctors and potentiators. METHODS: A Phase I program evaluated pharmacokinetics, drug-drug interactions and safety of cavosonstat in healthy and cystic fibrosis (CF) subjects homozygous for F508del-CFTR...
February 13, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28209107/gastro-intestinal-manifestations-in-cystic-fibrosis-patients
#15
REVIEW
S Van Biervliet, C de Clercq, D Declercq, E Van Braeckel, S Van Daele, F De Baets, D De Looze
Cystic fibrosis (CF) is a life-limiting disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR). This defective chloride channel, present in different organ systems such as respiratory system, gastrointestinal tract, reproductive system and sweat glands, disturbs the ion and water transport over the membranes leading to the well known CF symptoms. CF has outgrown paediatric care, as half of CF patients are currently adults. The CF gastrointestinal tract has its own particularities...
September 2016: Acta Gastro-enterologica Belgica
https://www.readbyqxmd.com/read/28208841/assessment-of-correlation-between-sweat-chloride-levels-and-clinical-features-of-cystic-fibrosis-patients
#16
Manzoor A Raina, Mosin S Khan, Showkat A Malik, Ab Hameed Raina, Mudassir J Makhdoomi, Javed I Bhat, Syed Mudassar
INTRODUCTION: Cystic Fibrosis (CF) is an autosomal recessive disorder and the incidence of this disease is undermined in Northern India. The distinguishable salty character of the sweat belonging to individuals suffering from CF makes sweat chloride estimation essential for diagnosis of CF disease. AIM: The aim of this prospective study was to elucidate the relationship of sweat chloride levels with clinical features and pattern of CF. MATERIALS AND METHODS: A total of 182 patients, with clinical features of CF were included in this study for quantitative measurement of sweat chloride...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28208047/targeted-therapy-for-chronic-respiratory-disease-a-new-paradigm
#17
Peter G Gibson, Matthew J Peters, Claire E Wainwright
Targeted therapy has emerged as a highly effective treatment approach for chronic respiratory diseases. Many of these conditions have dismal outcomes; however, targeted therapy shows great results for the subgroup who respond. This represents a new way to approach these conditions and offers great promise as a future treatment direction. In severe eosinophilic asthma, therapy that targets the interleukin-5 pathway with monoclonal antibodies leads to a 50% reduction in asthma exacerbations in previously refractory disease...
February 20, 2017: Medical Journal of Australia
https://www.readbyqxmd.com/read/28207476/faecal-calprotectin-in-cystic-fibrosis-and-it-s-relation-to-disease-parameters-a-longitudinal-analysis-over-12-years
#18
Helmut Ellemunter, Alexander Engelhardt, Katharina Schüller, Gratiana Steinkamp
OBJECTIVES: Faecal calprotectin (FC) is a marker of inflammation in the intestinal tract. We assessed FC levels longitudinally in patients with cystic fibrosis (CF) and evaluated the relation between FC results and relevant markers of disease. METHODS: Calprotectin was measured in faecal samples starting in 2003 and values were stored in the centre's patient database. In this retrospective analysis we searched for associations of FC concentrations with disease severity and progression...
February 15, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28204942/molecular-modeling-in-the-age-of-clinical-genomics-the-enterprise-of-the-next-generation
#19
Jeremy W Prokop, Jozef Lazar, Gabrielle Crapitto, D Casey Smith, Elizabeth A Worthey, Howard J Jacob
Protein modeling and molecular dynamics hold a unique toolset to aide in the characterization of clinical variants that may result in disease. Not only do these techniques offer the ability to study under characterized proteins, but they do this with the speed that is needed for time-sensitive clinical cases. In this paper we retrospectively study a clinical variant in the XIAP protein, C203Y, while addressing additional variants seen in patients with similar gastrointestinal phenotypes as the C203Y mutation...
March 2017: Journal of Molecular Modeling
https://www.readbyqxmd.com/read/28202251/high-incidence-of-non-tuberculous-mycobacteria-positive-cultures-among-adolescent-with-cystic-fibrosis
#20
Zoé Cavalli, Quitterie Reynaud, Romain Bricca, Raphaële Nove-Josserand, Stéphane Durupt, Philippe Reix, Marie Perceval, Michèle Pérouse de Montclos, Gérard Lina, Isabelle Durieu
BACKGROUND: We evaluated the prevalence of non-tuberculous mycobacteria (NTM)-positive cultures among our cystic fibrosis (CF) center patients, reviewed risk factors for NTM positivity, and determined its impact on lung function evolution. METHODS: From 2009 to 2014, CF adults and children attending the CF center of Lyon (France) and having at least one positive NTM isolate were included. Each case was matched by age and gender with two CF patients with no NTM isolate (controls)...
February 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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