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"Cystic fibrosis"

Paola Mosconi, Cinzia Colombo, Anna Roberto, Giulia Candiani, Maria Teresa Greco, Roberto Satolli, Carlo Castellani
Background: Health technology assessment and ethical issues have to be dealt with in deciding on national carrier screening for cystic fibrosis (CF)-the most frequent severe autosomal recessive disease in Caucasian populations and several stakeholders need to be involved. A citizens' jury is one way to ask citizens to deliberate on controversial topics in the interests of a society. The aims of this project were to gather opinions about CF carrier screening through citizens' jury deliberations and to match them with the findings of a large online consultation survey open to the general population, people with CF and families and health professionals...
March 19, 2018: European Journal of Public Health
Kelly E Wood
No abstract text is available yet for this article.
March 22, 2018: New England Journal of Medicine
J Manji, A Thamboo, M Tacey, C Garnis, N K Chadha
BACKGROUND: Sinonasal disease is a common feature of cystic fibrosis (CF) and can cause significant morbidity in these patients. Our objective was to determine if CF individuals with concomitant nasal polyposis (NP) express a unique profile of inflammation and if so, whether these inflammatory cytokine mediators have predictive value in identifying these individuals for prompt management by an Otolaryngologist. METHODOLOGY: Nasal lavage samples and clinical outcomes of disease severity were obtained from thirty-eight pediatric CF individuals...
March 21, 2018: Rhinology
Trey S Rottgen, Andrew J Nickerson, Emily A Minor, Amanda B Stewart, Abby D Harold, Vazhaikkurichi M Rajendran
Attenuated Ca2+ -activated Cl- secretion has previously been observed in the model of dextran-sulfate-sodium (DSS)-induced colitis. Prior studies have implicated dysfunctional muscarinic signaling from basolateral membranes as the potential perpetrator leading to decreased Ca2+ -activated Cl- secretion. However, in our chronic model of DSS-colitis, Chrm3 transcript (1.028 {plus minus} 0.12 vs. 1.029 {plus minus} 0.27, p > 0.05) and CHRM3 protein expression (1.021 {plus minus} .24 vs. .928 {plus minus} .09, p > 0...
March 21, 2018: American Journal of Physiology. Cell Physiology
Jozef Dingemans, Hanneke Eyns, Julie Willekens, Pieter Monsieurs, Rob Van Houdt, Pierre Cornelis, Anne Malfroot, Aurélie Crabbé
High levels of shear stress can prevent and disrupt Pseudomonas aeruginosa biofilm formation in vitro. Intrapulmonary percussive ventilation (IPV) could be used to introduce shear stress into the lungs of cystic fibrosis (CF) patients to disrupt biofilms in vivo. We performed a first-of-its-kind pilot clinical study to evaluate short-term IPV therapy at medium (200 bursts per minute, bpm) and high frequency (400 bpm) as compared to autogenic drainage (AD) on lung function and the behavior of P. aeruginosa in the CF lung in four patients who are chronically colonized by P...
March 20, 2018: European Journal of Clinical Microbiology & Infectious Diseases
Priya Kapoor, Philip Murphy
Objectives: To evaluate the effect of antibiotic combination therapy versus single therapy against cystic fibrosis strains of Pseudomonas aeruginosa identified as common and rare among patient groups in different Irish hospitals. Methods: This study compares the susceptibility profiles of P. aeruginosa isolates from different cystic fibrosis (CF) clinics in Ireland, collected from 2004-2005. Strains were recovered in small numbers and typed by pulsed-field gel electrophoresis...
March 2018: Heliyon
Elena K Schneider, Rachel M McQuade, Vincenzo C Carbone, Felisa Reyes-Ortega, John W Wilson, Brenda Button, Ayame Saito, Daniel P Poole, Daniel Hoyer, Jian Li, Tony Velkov
Ivacaftor-lumacaftor and ivacaftor are two new breakthrough cystic fibrosis transmembrane conductance modulators. The interactions of ivacaftor and its two metabolites hydroxymethylivacaftor (iva-M1) and ivacaftorcarboxylate (iva-M6) with neurotransmitter receptors were investigated in radioligand binding assays. Ivacaftor displayed significant affinity to the 5-hydroxytryptamine (5-HT; serotonin) 5-HT2C receptor (p K i =6.06±0.03), β3 -adrenergic receptor (p K i =5.71±0.07), δ-opioid receptor (p K i =5...
January 2018: ERJ Open Research
Maryam Hassanzad, Azam Orooji, Ali Valinejadi, Aliakbar Velayati
Background: Finding a valid diagnosis is mostly a prolonged process. Current advances in the sector of artificial intelligence have led to the appearance of expert systems that enrich the experiences and capabilities of doctors for making decisions for their patients. Objective: The objective of this research was developing a fuzzy expert system for diagnosing Cystic Fibrosis (CF). Methods: Defining the risk factors and then, designing the fuzzy expert system for diagnosis of CF were carried out in this cross-sectional study...
December 2017: Electronic Physician
Josselin Bodilis, Elodie Denet, Elisabeth Brothier, Arnault Graindorge, Sabine Favre-Bonté, Sylvie Nazaret
The Burkholderia cenocepacia epidemic ET12 lineage belongs to the genomovar IIIA including the reference strain J2315, a highly transmissible epidemic B. cenocepacia lineage. Members of this lineage are able to cause lung infections in immunocompromised and cystic fibrosis patients. In this study, we describe the genome of F01, an environmental B. cenocepacia strain isolated from soil in Burkina Faso that is, to our knowledge, the most closely related strain to this epidemic lineage. A comparative genomic analysis was performed on this new isolate, in association with five clinical and one environmental B...
2018: Frontiers in Microbiology
A Gramegna, B C Millar, F Blasi, J S Elborn, D G Downey, J E Moore
INTRODUCTION: Pulmonary exacerbations in people with Cystic Fibrosis (CF), with chronic Gram-negative pathogens, are associated with reduced survival. These pathogens are usually treated with repeated courses of systemic antibiotics. However there is a linked emergence of multidrug resistant (MDR) pathogens. Ceftolozane/tazobactam is a new cephalosporin/beta-lactamase inhibitor combination that has been demonstrated to have good activity against MDR Pseudomonas aeruginosa. MATERIAL & METHODS: In this study ceftolozane/tazobactam was compared to other commonly used intravenous antibiotics against 193 non-fermenting Gram-negative bacteria isolated from CF sputum specimens, including P...
March 17, 2018: Journal of Global Antimicrobial Resistance
Jonathan T Ma, Christina Tang, Le Kang, Judith A Voynow, Bruce K Rubin
BACKGROUND: Cystic fibrosis (CF) airway secretions are abnormal, contributing to decreased clearance and a cycle of infection and inflammation. CF sputum properties may predict disease progression. We hypothesized that sputum viscoelasticity and clearance abnormalities would inversely correlate with pulmonary function during exacerbation and that sputum properties would return to baseline after therapy. METHODS: We collected sputa longitudinally from 13 CF subjects with moderate to severe lung disease during both clinical stability and exacerbation...
March 17, 2018: Chest
Xiao Wei, Zongshi Lu, Tao Yang, Peng Gao, Sijiao Chen, Daoyan Liu, Zhiming Zhu
BACKGROUND/AIMS: High salt consumption is a major risk factor for hypertension, and sodium homeostasis is regulated by both intestinal sodium absorption and urinary sodium excretion. Chronic caffeine intake has been reported to attenuate salt-sensitive hypertension by promoting urinary sodium excretion; however, its exact role in intestinal sodium absorption remains unknown. Here, we investigated whether and how chronic caffeine consumption antagonizes salt-sensitive hypertension by inhibiting intestinal sodium absorption...
March 16, 2018: Kidney & Blood Pressure Research
Tamer Sekmenli, Metin Gündüz, Hikmet Akbulut, H Haldun Emiroglu, Mustafa Koplay, Ilhan Ciftci
Percutaneous endoscopic gastrostomy (PEG) is used as an alternative to enteral/nasoenteral feeding in situations where long-term oral feeding is ineffective or not tolerated. It is mostly preferred in patients with neurological conditions and also to support nutrition in patients with congenital heart diseases, cystic fibrosis, inflammatory bowel disease, and various oropharyngeal diseases. Although it is easily applicable compared to many invasive procedures, it has complications ranging from wound infection to death...
April 1, 2018: Archivos Argentinos de Pediatría
Candice Bjornson, Parco Chan, Abby Li, Bosco Paes, Krista L Lanctôt, Ian Mitchell
Respiratory syncytial virus (RSV) infection in cystic fibrosis (CF) infants is associated with significant morbidities. This study's objective is to evaluate the effectiveness and adverse events related to palivizumab (PVZ) in CF infants. Data on respiratory-related illness (RIH) and RSV hospitalizations (RSVH) were collected retrospectively in CF infants aged < 2 years in Alberta, Canada, from 2000 to 2017. Logistic regression models were used to compare the odds of RSVH or RIH in PVZ infants from the Canadian registry of palivizumab (CARESS) versus untreated (UPVZ) infants from Alberta, after adjusting for potential confounders...
March 19, 2018: European Journal of Clinical Microbiology & Infectious Diseases
K Schneider
The radiographic technique of pediatric chest X‑rays is substantially different from that in adults. In nearly all cases ap/pa X‑rays are sufficient and lateral radiographs are rarely needed. In the first years of life the thymus may overshadow the heart, the great vessels and the lung hila. The most important anatomical structures essential for diagnosing pathological findings of the lungs and mediastinum are the trachea with the bifurcation and the main bronchi with the adjacent great vessels. For the assessment of distended lungs and intrathoracic consolidations, fundamental knowledge of the anatomy in childhood and malformations which can involve the airways, the lungs, the heart, as well as systemic and pulmonary vessels are indispensable...
March 19, 2018: Der Radiologe
Emily K Cope
Despite growing efforts to understand the role of the microbiota in airway disease, mechanisms that link microbial community dysbiosis to chronic inflammation remain elusive. Our laboratory is interested in how altered microbiota composition or function influences airway inflammatory diseases, including chronic rhinosinusitis, asthma, and cystic fibrosis. Given the tight interplay between host-associated microbes and host immunity, the potential for translational microbiome research to guide clinical decisions and novel therapeutics is becoming better appreciated...
March 2018: MSystems
Xia Hou, Hongguang Wei, Carthic Rajagopalan, Hong Jiang, Qingtian Wu, Khalequz Zaman, Youming Xie, Fei Sun
Endoplasmic reticulum (ER)-associated protein degradation (ERAD) is an important quality control mechanism that eliminates misfolded proteins from the ER. The Derlin-1/VCP/VIMP protein complex plays an essential role in ERAD. Although the roles of Derlin-1 and VCP are relatively clear, the functional activity of VIMP in ERAD remains to be understood. Here we investigate the role of VIMP in the degradation of CFTRΔF508, a cystic fibrosis transmembrane conductance regulator (CFTR) mutant known to be a substrate of ERAD...
March 19, 2018: Scientific Reports
Thomas Gaisl, Lukas Bregy, Nina Stebler, Martin Thomas Gaugg, Tobias Bruderer, Diego García-Gómez, Alexander Moeller, Florian Singer, Esther Irene Schwarz, Christian Benden, Pablo M-L Sinues, Renato Zenobi, Malcolm Kohler
We aimed at defining profiles of volatile organic compounds in exhaled breath from patients with cystic fibrosis (CF) using a novel real-time mass spectrometry technique.&#13; In this prospective matched case-control study, 30 patients with CF, and 30 healthy control subjects were matched one-to-one according to age, gender, and smoking state. We performed exhaled breath analysis by untargeted secondary electrospray ionization-high resolution mass spectrometry (SESI-HRMS). &#13; Patients with CF (mean age 26...
March 20, 2018: Journal of Breath Research
Mohamed Salla, Rodrigo Aguayo-Ortiz, Danmaliki Gaddafi Ibrahim, Alaa Zare, Ahmed Said, Jack Moore, Vrajeshkumar Pandya, Robin Manaloor, Sunny Fong, Anna R Blankstein, Spencer Gibson, Laura Ramos Garcia, Pascal Meier, Khushwant S Bhullar, Basil P Hubbard, Yahya Fiteh, Harissios Vliagoftis, Ing Swie Goping, Dion Brocks, Peter Hwang, Jose Carlos A Martinez Velazquez, Shairaz Baksh
Receptor interacting protein kinase 2 (RIP2 or RICK herein referred to as RIPK2) is linked to the pathogen pathway that activates NFkB and autophagic activation. Using molecular modeling (docking) and chemoinformatics analyses we utilized the RIPK2/ponatinib crystal structure and searched in chemical databases for small molecules exerting binding interactions similar to those exerted by ponatinib. The identified RIPK2 inhibitors potently inhibited the proliferation of cancer cells by > 70% as well as inhibition of NFkB activity...
March 19, 2018: Journal of Pharmacology and Experimental Therapeutics
Francesca Forti, Dwayne R Roach, Marco Cafora, Maria E Pasini, David S Horner, Ersilia V Fiscarelli, Martina Rossitto, Lisa Cariani, Federica Briani, Laurent Debarbieux, Daniela Ghisotti
The alarming diffusion of multidrug resistant (MDR) bacterial strains requires investigations on non-antibiotic therapies. Amongst them, the use of bacteriophages (phages) as antimicrobial agents, namely phage therapy, is a promising treatment strategy with support by recent successful compassionate treatments in Europe and the U.S.A. In this work, we combined host range and genomic information to design a 6-phage cocktail killing several clinical strains of P. aeruginosa , including those collected from Italian cystic fibrosis (CF) patients, and analyzed the cocktail performance...
March 19, 2018: Antimicrobial Agents and Chemotherapy
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