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Cystic Fibrosis

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https://www.readbyqxmd.com/read/29058126/time-kill-analysis-of-ceftolozane-tazobactam-efficacy-against-mucoid-pseudomonas-aeruginosa-strains-from-cystic-fibrosis-patients
#1
Hana Rac, Kayla R Stover, Jamie L Wagner, S Travis King, Henderson D Warnock, Katie E Barber
INTRODUCTION: Mucoid Pseudomonas aeruginosa (MP) strains in cystic fibrosis (CF) patients are thought to initiate the chronic infection stage of CF and are associated with pulmonary function decline. OBJECTIVES: The purpose of this study was to assess the susceptibility of MP strains to ceftolozane/tazobactam and the efficacy of ceftolozane/tazobactam against MP strains compared with those for standard-of-care antipseudomonal antibiotics. METHODS: Ten clinical isolates of MP from CF patients were tested for susceptibility with Etest and time-kill analysis with ceftolozane/tazobactam compared with ceftazidime, cefepime, ciprofloxacin, meropenem, tobramycin, and polymyxin B...
October 20, 2017: Infectious Diseases and Therapy
https://www.readbyqxmd.com/read/29057987/magnetic-resonance-imaging-features-of-dogs-with-incomplete-recovery-after-acute-severe-spinal-cord-injury
#2
Melissa J Lewis, Eli B Cohen, Natasha J Olby
STUDY DESIGN: Retrospective case series. OBJECTIVES: Describe the magnetic resonance imaging (MRI) features of dogs chronically impaired after severe spinal cord injury (SCI) and investigate associations between imaging variables and residual motor function. SETTING: United States of America. METHODS: Thoracolumbar MRI from dogs with incomplete recovery months to years after clinically complete (paralysis with loss of pain perception) thoracolumbar SCI were reviewed...
October 23, 2017: Spinal Cord
https://www.readbyqxmd.com/read/29056926/highly-diversified-pandoraea-pulmonicola-population-during-chronic-colonization-in-cystic-fibrosis
#3
Chloé Dupont, Fabien Aujoulat, Raphaël Chiron, Pauline Condom, Estelle Jumas-Bilak, Hélène Marchandin
Several environmental bacteria are considered as opportunistic pathogens in cystic fibrosis (CF) and are able to persistently colonize the CF respiratory tract (CFRT). Beside Pseudomonas aeruginosa and Burkholderia cepacia complex, Pandoraea spp. are defined as pathogenic. During chronic colonization, adaptive evolution and diversified population have been demonstrated, notably for P. aeruginosa. However, the persistence of Pandoraea in the CFRT remains largely unexplored. We studied genomic and phenotypic traits of Pandoraea pulmonicola isolates successively recovered from the airways of a single CF patient and relate the results to qualitative and quantitative evolution of other cultivable pathogens and to patient clinical status...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/29056478/survival-of-pathogenic-m-abscesssus-subsp-massiliense-in-acanthamoeba-castellanii
#4
J L DaSilva, Jan Nguyen, Kevin P Fennelly, Adrian M Zelazny, Kenneth N Olivier
We used an amoeba model to study the intracellular growth and cytotoxicity of clinical strains of M. abscessus subsp. massiliense (Mabsm) isolated from 2 patients (one with cystic fibrosis, the other one with idiopathic bronchiectasis) during the early (smooth colonies) and late stage (rough colonies) of chronic pulmonary infection. Acanthamoeba castellanii were infected with Mabsm (MOI 100) and samples collected every 24h for 72 h. Results showed Mabsm is able to survive in trophozoites and persist in cysts for at least 7 days...
October 19, 2017: Research in Microbiology
https://www.readbyqxmd.com/read/29056429/three-dimensional-microbiome-and-metabolome-cartography-of-a-diseased-human-lung
#5
Neha Garg, Mingxun Wang, Embriette Hyde, Ricardo R da Silva, Alexey V Melnik, Ivan Protsyuk, Amina Bouslimani, Yan Wei Lim, Richard Wong, Greg Humphrey, Gail Ackermann, Timothy Spivey, Sharon S Brouha, Nuno Bandeira, Grace Y Lin, Forest Rohwer, Douglas J Conrad, Theodore Alexandrov, Rob Knight, Pieter C Dorrestein
Our understanding of the spatial variation in the chemical and microbial makeup of an entire human organ remains limited, in part due to the size and heterogeneity of human organs and the complexity of the associated metabolome and microbiome. To address this challenge, we developed a workflow to enable the cartography of metabolomic and microbiome data onto a three-dimensional (3D) organ reconstruction built off radiological images. This enabled the direct visualization of the microbial and chemical makeup of a human lung from a cystic fibrosis patient...
October 17, 2017: Cell Host & Microbe
https://www.readbyqxmd.com/read/29056168/cystic-fibrosis-a-risk-condition-for-renal-disease
#6
Domenico Santoro, Adele Postorino, Cristina Lucanto, Stefano Costa, Simona Cristadoro, Salvatore Pellegrino, Giovanni Conti, Michele Buemi, Giuseppe Magazzù, Guido Bellinghieri
OBJECTIVE: Cystic fibrosis (CF) is the most common autosomal recessive disease affecting the Caucasian population, with a birth incidence ranging between 1:2,500 and 1:1,800. It is caused by mutations in the CF transmembrane regulator gene which is localized on 7 chromosomes. Renal disease is reported as a relatively rare complication in adult patient with CF. We evaluated proteinuria and chronic renal failure (CRF) in a population of patients with CF. METHODS: A retrospective study was carried out in a referral center for CF at University of Messina in Italy...
November 2017: Journal of Renal Nutrition
https://www.readbyqxmd.com/read/29055982/vitamin-a-status-and-its-determinants-in-patients-with-cystic-fibrosis
#7
Ewa Sapiejka, Patrycja Krzyżanowska, Dariusz Walkowiak, Ewa Wenska-Chyży, Mariusz Szczepanik, Szczepan Cofta, Andrzej Pogorzelski, Wojciech Skorupa, Jarosław Walkowiak
BACKGROUND: Routine administration of vitamin A, recommended in CF patients, can help to prevent its deficiency. However, high vitamin A supplementation may lead to its excessive level and possible toxicity. Therefore, the aim of the present study was to assess the status of vitamin A and the determinants of its body resources in CF patients. METHODS: In 196 CF patients aged from 4 months to 47 years, the following parameters  were analysed: nutritional status (standardized body weight and height, serum albumin concentration) and clinical expression of disease (lung function - spirometry; biochemical markers of liver function - ALT, AST, GGT; respiratory tract colonization by Pseudomonas aeruginosa; diabetes; cirrhosis, non-cirrhotic liver disease; exocrine pancreatic function - fecal elastase-1 concentration; blood clotting -  INR  and  vitamin  A supplementation)...
July 2017: Acta Scientiarum Polonorum. Technologia Alimentaria
https://www.readbyqxmd.com/read/29055510/prevalence-of-burkholderia-cepacia-complex-species-in-cystic-fibrosis-patients-in-argentina-during-the-period-2011-2015
#8
Lucía Cipolla, Florencia Rocca, Claudia Martinez, Lorena Aguerre, Rubén Barrios, Mónica Prieto
INTRODUCTION: Burkholderia cepacia (B. cepacia) complex is composed of 20 phylogenetically closely related bacterial species. Some species have emerged as opportunistic pathogens in immunocompromised patients and are responsible for nosocomial outbreaks. The B. cepacia complex is a recognized respiratory pathogen in patients with cystic fibrosis. Burkholderia cenocepacia and Burkholderia multivorans (B. multivorans) are the most prevalent species in the world, according to the literature...
October 18, 2017: Enfermedades Infecciosas y Microbiología Clínica
https://www.readbyqxmd.com/read/29055499/singing-as-an-adjunct-therapy-for-children-and-adults-with-cystic-fibrosis
#9
Lisa Whitehead, R N Diana Arabiat, Mandie Foster
No abstract text is available yet for this article.
September 15, 2017: International Journal of Nursing Studies
https://www.readbyqxmd.com/read/29055397/vitamin-k-deficit-and-elastolysis-theory-in-pulmonary-elasto-degenerative-diseases
#10
Rob Janssen, Cees Vermeer
Elastin is a unique protein providing deformability and resilience to dynamic tissues, such as arteries and lungs. It is an absolute basic requirement for circulation and respiration. Elastin can be degraded by elastases and has a high calcium affinity. Elastin calcification and elastin degradation are two pathological processes that impair elastin's functioning. Furthermore, elastin degradation can be associated to elastin calcification. Matrix Gla Protein (MGP) is probably the most potent natural inhibitor of elastin calcification and requires vitamin K for its activation...
October 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/29055231/correcting-cftr-folding-defects-by-small-molecule-correctors-to-cure-cystic-fibrosis
#11
REVIEW
Marjolein Mijnders, Bertrand Kleizen, Ineke Braakman
Pharmacological intervention to treat the lethal genetic disease cystic fibrosis has become reality, even for the severe, most common folding mutant F508del CFTR. CFTR defects range from absence of the protein, misfolding that leads to degradation rather than cell-surface localization (such as F508del), to functional chloride-channel defects on the cell surface. Corrector and potentiator drugs improve cell-surface location and channel activity, respectively, and combination therapy of two correctors and a potentiator have shown synergy...
October 18, 2017: Current Opinion in Pharmacology
https://www.readbyqxmd.com/read/29054758/cystic-fibrosis-transmembrane-regulator-haplotypes-in-households-of-patients-with-cystic-fibrosis
#12
Daniela Tenório Furgeri, Fernando Augusto Lima Marson, Cyntia Arivabeni Araújo Correia, José Dirceu Ribeiro, Carmen Sílvia Bertuzzo
INTRODUCTION: Nearly 2000 mutations in the cystic fibrosis transmembrane regulator (CFTR) gene have been reported. The F508del mutation occurs in approximately 50-65% of patients with cystic fibrosis (CF). However, molecular diagnosis is not always possible. Therefore, silent polymorphisms can be used to label the mutant allele in households of patients with CF. OBJECTIVE: To verify the haplotypes of four polymorphisms at the CFTR locus in households of patients with CF for pre-fertilization, pre-implantation, and prenatal indirect mutation diagnosis to provide better genetic counseling for families and patients with CF and to associate the genotypes/haplotypes with the F508del mutation screening...
October 17, 2017: Gene
https://www.readbyqxmd.com/read/29053845/substitution-of-yor1p-nbd1-residues-improves-the-thermal-stability-of-human-cystic-fibrosis-transmembrane-conductance-regulator
#13
B M Xavier, E Hildebrandt, F Jiang, H Ding, J C Kappes, I L Urbatsch
The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) is a plasma membrane chloride channel protein that regulates vertebrate fluid homeostasis. The inefficiency of wild type human CFTR protein folding/trafficking is exacerbated by genetic mutations that can cause protein misfolding in the endoplasmic reticulum (ER) and subsequent degradation. This project investigates small changes in protein sequence that can alter the thermal stability of the large multi-domain CFTR protein. We target a conserved 70-residue α-subdomain located in the first nucleotide-binding domain that hosts the common misfolding mutation ∆F508...
October 19, 2017: Protein Engineering, Design & Selection: PEDS
https://www.readbyqxmd.com/read/29053438/tailored-approach-to-surgical-exposure-reduces-surgical-site-complications-after-bilateral-lung-transplantation
#14
Stefan Elde, Stephen Huddleston, Scott Jackson, Rosemary Kelly, Sara Shumway, Gabriel Loor
BACKGROUND: We evaluated the effects of tailoring the operative approach on major surgical site complications and outcomes in lung transplant recipients. PATIENTS AND METHODS: Beginning in July 2013, bilateral lung transplants at a single institution were performed either through sternotomy or clamshell depending on proximity of hilar structures by computed tomography (CT), anticipated complexity, past surgical history, and surgeon experience. Patient demographics and outcomes were collected in the institution's Transplant Information Services (TIS)...
October 20, 2017: Surgical Infections
https://www.readbyqxmd.com/read/29053080/aerosolizing-lipid-dispersions-enables-antibiotic-transport-across-mimics-of-the-lung-airway-surface-even-in-the-presence-of-pre-existing-lipid-monolayers
#15
Steven V Iasella, Amy Z Stetten, Timothy E Corcoran, Stephen Garoff, Todd M Przybycien, Robert D Tilton
BACKGROUND: Secondary lung infections are the primary cause of morbidity associated with cystic fibrosis lung disease. Aerosolized antibiotic inhalation is potentially advantageous but has limited effectiveness due to altered airway aerodynamics and deposition patterns that limit drug access to infected regions. One potential strategy to better reach infected areas is to formulate aerosols with surfactants that induce surface tension gradients and drive postdeposition drug dispersal via Marangoni transport along the airway surface liquid (ASL)...
October 20, 2017: Journal of Aerosol Medicine and Pulmonary Drug Delivery
https://www.readbyqxmd.com/read/29052582/idiopathic-pulmonary-fibrosis
#16
REVIEW
Fernando J Martinez, Harold R Collard, Annie Pardo, Ganesh Raghu, Luca Richeldi, Moises Selman, Jeffrey J Swigris, Hiroyuki Taniguchi, Athol U Wells
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease characterized by progressive lung scarring and the histological picture of usual interstitial pneumonia (UIP). It is associated with increasing cough and dyspnoea and impaired quality of life. IPF affects ∼3 million people worldwide, with incidence increasing dramatically with age. The diagnostic approach includes the exclusion of other interstitial lung diseases or overlapping conditions and depends on the identification of the UIP pattern, usually with high-resolution CT; lung biopsy might be required in some patients...
October 20, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/29048768/family-centred-care-for-families-living-with-cystic-fibrosis-in-a-rural-setting-a-qualitative-study
#17
Melanie Jessup, Wendy Smyth, Gail Abernethy, Linda Shields, Tonia Douglas
AIMS AND OBJECTIVES: This study aimed to explore experience of family-centred care among parents of children with cystic fibrosis living far from tertiary treatment centres and to understand what such distances mean to their care. BACKGROUND: Australia is a large continent. However, many families with a child with cystic fibrosis live in regional areas, often thousands of kilometres away from the primary treatment centres located in Australia's coastal capital cities...
October 19, 2017: Journal of Clinical Nursing
https://www.readbyqxmd.com/read/29048473/use-of-taurolidine-in-lung-transplantation-for-cystic-fibrosis-and-impact-on-bacterial-colonization
#18
Mohamed Zeriouh, Anton Sabashnikov, Nikhil P Patil, Bastian Schmack, Barlomiej Zych, Prashant N Mohite, Diana García Sáez, Achim Koch, Ashham Mansur, Simona Soresi, Alexander Weymann, Nandor Marczin, Thorsten Wahlers, Fabio De Robertis, André Rüdiger Simon, Aron-Frederik Popov
OBJECTIVES: The presence of bacterial colonization that causes chronic pulmonary infections in cystic fibrosis (CF) patients remains a key issue before lung transplantation. We sought to assess the impact of intraoperative taurolidine lavage on bacterial colonization and long-term outcomes following lung transplantation in CF patients. METHODS: Between 2007 and 2013, 114 CF patients underwent lung transplantation at our institute, and taurolidine 2% bronchial lavage was applied in a substantial proportion of patients (n = 42)...
October 18, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29046368/enhancement-of-lung-gene-delivery-after-aerosol-a-new-strategy-using-non-viral-complexes-with-antibacterial-properties
#19
Angelique Mottais, Tony Le Gall, Yann Sibiril, Julian Ravel, Véronique Laurent, Frédérique d'Arbonneau, Tristan Montier
The pathophysiology of obstructive pulmonary diseases, such as cystic fibrosis, leads to the development of chronic infections in the respiratory tract. Thus, the symptomatic management of the disease requires, in particular, repetitive antibiotherapy. Besides these antibacterial treatments, certain pathologies, such as cystic fibrosis or chronic obstructive pulmonary disease, require the intake of many drugs. This simultaneous absorption may lead to undesired drug interactions. For example, Orkambi® (lumacaftor/Ivacaftor, Vertex), a pharmacological drug employed to treat F508del patients, cannot be used with antibiotics such as rifampicin or rifabutin (rifamycin family) which are necessary to treat Mycobacteriaceae...
October 18, 2017: Bioscience Reports
https://www.readbyqxmd.com/read/29045949/effectiveness-of-a-home-based-active-video-game-programme-in-young-cystic-fibrosis-patients
#20
Tamara Del Corral, Maria Àngels Cebrià I Iranzo, Ibai López-de-Uralde-Villanueva, Roberto Martínez-Alejos, Isabel Blanco, Jordi Vilaró
BACKGROUND: Exercise-based rehabilitation is already a part of cystic fibrosis (CF) treatment; however, patient adherence is low. OBJECTIVES: To assess the effectiveness of a home exercise programme using active video games (AVGs) as a training modality for children and adolescents with CF. METHODS: Thirty-nine children with CF were randomised to a control group (CG, n = 20, age 11 ± 6 years; FEV1 86.2 ± 20.5% of predicted) or a training group (AVGG, n = 19, age 13 ± 3 years; FEV1 82...
October 19, 2017: Respiration; International Review of Thoracic Diseases
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