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Cystic fibrosis

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https://www.readbyqxmd.com/read/29333815/prevalence-of-vitamin-k-deficiency-and-associated-factors-in-non-supplemented-cystic-fibrosis-patients
#1
Patrycja Krzyżanowska, Sławomira Drzymala-Czyż, Nataliya Rohovyk, Lyudmyla Bober, Jerzy Moczkco, Marta Rachel, Jarosław Walkowiak
INTRODUCTION: Vitamin K deficiency is highly prevalent in cystic fibrosis (CF) patients despite supplementation. Moreover, no reliable risk factors for its occurrence are known. The aim was to assess the prevalence of vitamin K deficiency and associated factors in non-supplemented CF patients. METHODS: Prothrombin concentration induced by vitamin K absence (PIVKA-II) and the undercarboxylated osteocalcin percentage (u-OC) were determined. In all patients clinical status was assessed and its relation to vitamin K deficiency determined...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333432/cytomegalovirus-associated-pulmonary-exacerbation-in-patients-with-cystic-fibrosis
#2
Akhil Sawant, Giulia Spoletini, Paul Whitaker, Christine Etherington, Ian Clifton, Daniel Peckham
CMV is an unusual cause of pulmonary exacerbation in immunocompetent individuals with CF http://ow.ly/Rdds30hlnjV.
January 2018: ERJ Open Research
https://www.readbyqxmd.com/read/29332976/australasian-guideline-2nd-edition-an-annex-to-the-clsi-and-uk-guidelines-for-the-performance-of-the-sweat-test-for-the-diagnosis-of-cystic-fibrosis
#3
John Massie, Ronda Greaves, Michael Metz, Veronica Wiley, Peter Graham, Samantha Shepherd, Richard Mackay
No abstract text is available yet for this article.
November 2017: Clinical Biochemist. Reviews
https://www.readbyqxmd.com/read/29332379/correlation-among-atherosclerosis-cardiac-and-respiratory-function-in-subjects-with-cystic-fibrosis-a-pilot-study
#4
Marco M Ciccone, Francesca Cortese, Michele Gesualdo, Pietro Scicchitano, Gabriella Ricci, Santa Carbonara, Michele Milo, Rosa Carbonara, Flavia Urbano, Anna M Cortese, Antonio Vergori, Antonio Manca, Luigi Mappa
No abstract text is available yet for this article.
January 11, 2018: Minerva Medica
https://www.readbyqxmd.com/read/29332002/the-implementation-of-a-cystic-fibrosis-annual-review-process-in-a-tertiary-paediatric-hospital
#5
Raffaela Armiento, Sarath C Ranganathan, Joanne Harrison
We evaluated the implementation of a cystic fibrosis annual review process in a tertiary paediatric hospital. After implementation, there was demonstrated improvement in an important outcome measure-the use of inhaled mucolytic agents.
January 13, 2018: Archives of Disease in Childhood. Education and Practice Edition
https://www.readbyqxmd.com/read/29330966/static-hyperinflation-is-associated-with-ventilatory-limitation-and-exercise-tolerance-in-adult-cystic-fibrosis
#6
Daniel Stevens
INTRODUCTION: Lung hyperinflation is a potential mechanism limiting exercise tolerance. However, available data on the impact of static hyperinflation on exercise performance in adult cystic fibrosis is lacking. Furthermore, the relative contribution of both static and dynamic hyperinflation to exercise performance is unknown. OBJECTIVES: To determine the impact of static hyperinflation on exercise tolerance and lung dynamics in adult cystic fibrosis. METHODS: Clinical data of 107 adult patients with cystic fibrosis, including pulmonary function, lung volumes, and cardiopulmonary exercise from the Toronto Cystic Fibrosis database were collected and analyzed...
January 13, 2018: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/29329721/quantifying-fluctuation-in-glucose-levels-to-identify-early-changes-in-glucose-homeostasis-in-cystic-fibrosis
#7
Rossa Brugha, Marie Wright, Suzie Nolan, Nicola Bridges, Siobhán B Carr
BACKGROUND: Cystic fibrosis related diabetes (CFRD) is associated with increased morbidity in CF. Variability in physiological systems is associated with dysfunctional homeostasis. We examined whether fluctuation in glucose is a marker of CFRD or "pre-diabetes". METHODS: Using a machine learning approach, we compared glucose IQR to current diagnostic criteria in a review of continuous glucose monitoring data. RESULTS: Analysis was performed on 248 studies from 142 children...
January 9, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29329667/gel-forming-mucin-interactome-drives-mucus-viscoelasticity
#8
REVIEW
Bastien Demouveaux, Valérie Gouyer, Frédéric Gottrand, Tetsuharu Narita, Jean-Luc Desseyn
Mucus is a hydrogel that constitutes the first innate defense in all mammals. The main organic component of mucus, gel-forming mucins, forms a complex network through both reversible and irreversible interactions that drive mucus gel formation. Significant advances in the understanding of irreversible gel-forming mucins assembly have been made using recombinant protein approaches. However, little is known about the reversible interactions that may finely modulate mucus viscoelasticity, which can be characterized using rheology...
December 28, 2017: Advances in Colloid and Interface Science
https://www.readbyqxmd.com/read/29329392/risk-of-pancreatic-cancer-associated-with-family-history-of-cancer-and-other-medical-conditions-by-accounting-for-smoking-among-relatives
#9
E Molina-Montes, P Gomez-Rubio, M Márquez, M Rava, M Löhr, C W Michalski, X Molero, A Farré, J Perea, W Greenhalf, L Ilzarbe, M O'Rorke, A Tardón, T Gress, V M Barberà, T Crnogorac-Jurcevic, E Domínguez-Muñoz, L Muñoz-Bellvís, J Balsells, E Costello, J Huang, M Iglesias, J Kleeff, Bo Kong, J Mora, L Murray, D O'Driscoll, I Poves, A Scarpa, W Ye, M Hidalgo, L Sharp, A Carrato, F X Real, N Malats
Background: Family history (FH) of pancreatic cancer (PC) has been associated with an increased risk of PC, but little is known regarding the role of inherited/environmental factors or that of FH of other comorbidities in PC risk. We aimed to address these issues using multiple methodological approaches. Methods: Case-control study including 1431 PC cases and 1090 controls and a reconstructed-cohort study (N = 16 747) made up of their first-degree relatives (FDR)...
January 10, 2018: International Journal of Epidemiology
https://www.readbyqxmd.com/read/29327948/effects-of-lumacaftor-ivacaftor-therapy-on-cftr-function-in-phe508del-homozygous-patients-with-cystic-fibrosis
#10
Simon Y Graeber, Christian Dopfer, Lutz Naehrlich, Lena Gyulumyan, Heike Scheuermann, Stephanie Hirtz, Sabine Wege, Heimo Mairbäurl, Marie Dorda, Rebecca Hyde, Azadeh Bagheri-Hanson, Claudia Rueckes-Nilges, Sebastian Fischer, Marcus A Mall, Burkhard Tümmler
RATIONALE: The combination of the CFTR corrector lumacaftor with the potentiator ivacaftor has been approved for the treatment of patients with cystic fibrosis (CF) homozygous for the Phe508del CFTR mutation. The phase 3 trials examined clinical outcomes, but did not evaluate CFTR function in patients. OBJECTIVES: To examine the effect of lumacaftor-ivacaftor on biomarkers of CFTR function in Phe508del homozygous CF patients aged 12 years and older. METHODS: This prospective observational study assessed clinical outcomes including FEV1 % predicted and BMI, and CFTR biomarkers including sweat chloride concentration, nasal potential difference (NPD) and intestinal current measurement (ICM) before and 8-16 weeks after initiation of lumacaftor-ivacaftor...
January 12, 2018: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29327941/infection-is-not-required-for-mucoinflammatory-lung-disease-in-cftr-knockout-ferrets
#11
Bradley H Rosen, T Idil Apak Evans, Shashanna R Moll, Jaimie S Gray, Bo Liang, Xingshen Sun, Yulong Zhang, Chandler W Jensen-Cody, Anthony M Swatek, Weihong Zhou, Nan He, Pavana G Rotti, Scott R Tyler, Nicholas W Keiser, Preston J Anderson, Leonard Brooks, Yalan Li, R Marshall Pope, Maheen Rajput, Eric A Hoffman, Kai Wang, J Kirk Harris, Kalpaj R Parekh, Katherine N Gibson-Corley, John F Engelhardt
RATIONALE: Classical interpretation of cystic fibrosis (CF) lung disease pathogenesis suggests that infection initiates disease progression, leading to an exuberant inflammatory response, excessive mucus, and ultimately bronchiectasis. While symptomatic antibiotic treatment controls lung infections early in disease, life-long bacterial residence typically ensues. Processes that control the establishment of persistent bacteria in the CF lung, and the contribution of non-infectious components to disease pathogenesis, are poorly understood...
January 12, 2018: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29326970/revisiting-sweat-chloride-test-results-based-on-recent-guidelines-for-diagnosis-of-cystic-fibrosis
#12
Jayson V Pagaduan, Mahesheema Ali, Michael Dowlin, Liye Suo, Tabitha Ward, Fadel Ruiz, Sridevi Devaraj
Objectives: Recent sweat chloride guidelines published by the Cystic Fibrosis Foundation changed the intermediate sweat chloride concentration range from 40-59 mmol/L to 30-59 mmol/L for age > 6 months. We wanted to know how this new guideline would impact detection of cystic fibrosis among patients who previously had sweat tests done at Texas Children's Hospital. Methods: We revisited sweat chloride test results (n = 3012) in the last 5 years at Texas Children's Hospital based on the new guidelines on diagnosis of cystic fibrosis from the Cystic Fibrosis Foundation...
March 2018: Practical Laboratory Medicine
https://www.readbyqxmd.com/read/29326226/draft-genome-sequence-of-chryseobacterium-mucoviscidosis-sp-nov-strain-vt16-26-isolated-from-the-bronchoalveolar-lavage-fluid-of-a-patient-with-cystic-fibrosis
#13
George Tetz, Victor Tetz
We report here the draft genome sequence of Chryseobacterium mucoviscidosis VT16-26, a novel bacterium isolated from the lungs of a patient with cystic fibrosis. The genome was composed of 4,403,956 bp and had 36.2% G+C content. We detected 4,048 genes with predicted protein-coding functions, including those associated with antibiotic resistance and virulence.
January 11, 2018: Genome Announcements
https://www.readbyqxmd.com/read/29326073/retinoic-acid-promotes-stem-cell-differentiation-and-embryonic-development-by-transcriptionally-activating-cftr
#14
Xiaofeng Li, Kin Lam Fok, Jinghui Guo, Yan Wang, Zhenqing Liu, Ziyi Chen, Chengdong Wang, Ye Chun Ruan, Sidney Siubun Yu, Hui Zhao, Ji Wu, Xiaohua Jiang, Hsiao Chang Chan
Retinoic acid (RA) plays a pivotal role in many cellular processes; however, the signaling mechanisms mediating the effect of RA are not fully understood. Here, we show that RA transcriptionally upregulates cystic fibrosis transmembrane conductance regulator (Cftr) by promoting the direct binding of its receptor RARα to Cftr promoter in mouse spermatogonia and embryonic stem (ES) cells. The RA/CFTR pathway is involved in the differentiation of spermatogonia and organogenesis during the embryo development of Xenopus laevis...
January 8, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29325044/adaptation-of-staphylococcus-aureus-to-the-airways-of-cystic-fibrosis-patients-by-the-up-regulation-of-superoxide-dismutase-m-and-iron-scavenging-proteins
#15
Janina Treffon, Desiree Block, Martin Moche, Swantje Reiß, Stephan Fuchs, Susanne Engelmann, Dörte Becher, Lars Langhanki, Alexander Mellmann, Georg Peters, Barbara C Kahl
Staphylococcus aureus represents an important pathogen in cystic fibrosis (CF) caused by early and persistent colonization of airways of these patients for extended periods. Although the median predicted survival age of CF patients has increased during the last decade, pulmonary infections are still a challenge.In this study we investigated the adaptation of S. aureus, which was cultured from the airways of an individual CF patient, by comparing the proteome of the first and latest available isolate recovered 13 years apart...
January 6, 2018: Journal of Infectious Diseases
https://www.readbyqxmd.com/read/29324908/peripheral-quantitative-computed-tomography-detects-differences-at-the-radius-in-prepubertal-children-with-cystic-fibrosis-compared-to-healthy-controls
#16
Catherine E O'Brien, Gulnur Com, John Fowlkes, Xinyu Tang, Laura P James
INTRODUCTION: In 2015, 11.9% of people with cystic fibrosis (CF) in the United States had osteopenia, 5.1% osteoporosis, and 0.3% experienced a fracture. Screening for CF-related bone disease starts in childhood, and dual energy x-ray absorptiometry (DXA) is the recommended method. It is unknown whether peripheral quantitative computed tomography (pQCT) can detect deficits earlier than DXA. This study compared pQCT and DXA scans in a group of pre-pubertal children with CF and healthy controls...
2018: PloS One
https://www.readbyqxmd.com/read/29323929/variation-in-cilia-protein-genes-and-progression-of-lung-disease-in-cystic-fibrosis
#17
Elizabeth Blue, Tin L Louie, Jessica X Chong, Scott J Hebbring, Kathleen C Barnes, Nicholas M Rafaels, Michael R Knowles, Ronald L Gibson, Michael J Bamshad, Mary J Emond
RATIONALE: Cystic fibrosis, like primary ciliary dyskinesia, is an autosomal recessive disorder characterized by abnormal mucociliary clearance and obstructive lung disease. We hypothesized that genes underlying the development or function of cilia may modify lung disease severity in persons with cystic fibrosis. OBJECTIVES: To test this hypothesis, we compared variants in 93 candidate genes in both upper and lower tertiles of lung function in a large cohort of children and adults with cystic fibrosis to a population control data set...
January 11, 2018: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29323796/treatment-of-chronic-rhinosinusitis-with-dornase-alfa-in-patients-with-cystic-fibrosis-a-systematic-review
#18
Gopi B Shah, Linde De Keyzer, Joy A Russell, Ashleigh Halderman
BACKGROUND: A major component of sputum in cystic fibrosis (CF) patients is polymerized DNA, a byproduct of degraded neutrophils. Dornase alfa (dornase) selectively cleaves extracellular DNA and reduces the viscosity of sputum. It improves mucociliary clearance and pulmonary function. The benefit of dornase on CF-associated sinusitis is less clear. Therefore, the objective of this study was to systematically review the use of dornase on chronic rhinosinusitis (CRS) in CF patients. METHODS: The Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement was followed for this systematic review...
January 11, 2018: International Forum of Allergy & Rhinology
https://www.readbyqxmd.com/read/29323060/spectrum-of-hepatobiliary-cystic-lesions-a-7-year-experience-at-a-tertiary-care-referral-center-in-north-india-and-review-of-literature
#19
Prasenjit Das, Pragya Sharma, Tripti Nakra, Shouriyo Ghosh, Rajni Yadav, Brijnandan Gupta, Gaurav Khanna, K S Madhusudhan, Rajesh Panwar, M K Anoop, Ragini Kilambi, Anand N Singh, Nihar R Dash, Sujoy Pal, Siddhartha Datta Gupta
CONTEXT: Cysts arising from the hepatobiliary tree are a group of heterogeneous lesions with regard to pathogenesis, clinical presentation, and radiological finding. They can be intrahepatic or extrahepatic, developmental, secondary to infective/inflammatory etiologies, as well as neoplastic. This study was conducted to determine the spectrum of hepatobiliary cysts in surgically intervened cases, with regard to their prevalence, histological spectrum, and clinicoradiological correlation, wherever possible...
October 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29322632/approaches-to-sample-size-calculation-for-clinical-trials-in-rare-diseases
#20
Frank Miller, Sarah Zohar, Nigel Stallard, Jason Madan, Martin Posch, Siew Wan Hee, Michael Pearce, Mårten Vågerö, Simon Day
We discuss 3 alternative approaches to sample size calculation: traditional sample size calculation based on power to show a statistically significant effect, sample size calculation based on assurance, and sample size based on a decision-theoretic approach. These approaches are compared head-to-head for clinical trial situations in rare diseases. Specifically, we consider 3 case studies of rare diseases (Lyell disease, adult-onset Still disease, and cystic fibrosis) with the aim to plan the sample size for an upcoming clinical trial...
January 10, 2018: Pharmaceutical Statistics
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