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Cystic Fibrosis

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https://www.readbyqxmd.com/read/28108847/airway-mucus-inflammation-and-remodeling-emerging-links-in-the-pathogenesis-of-chronic-lung-diseases
#1
REVIEW
Zhe Zhou-Suckow, Julia Duerr, Matthias Hagner, Raman Agrawal, Marcus A Mall
Airway mucus obstruction is a hallmark of many chronic lung diseases including rare genetic disorders such as cystic fibrosis (CF) and primary ciliary dyskinesia, as well as common lung diseases such as asthma and chronic obstructive pulmonary disease (COPD), which have emerged as a leading cause of morbidity and mortality worldwide. However, the role of excess airway mucus in the in vivo pathogenesis of these diseases remains poorly understood. The generation of mice with airway-specific overexpression of epithelial Na(+) channels (ENaC), exhibiting airway surface dehydration (mucus hyperconcentration), impaired mucociliary clearance (MCC) and mucus plugging, led to a model of muco-obstructive lung disease that shares key features of CF and COPD...
January 20, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/28108166/predictive-performance-of-different-kidney-function-estimation-equations-in-lung-transplant-patients
#2
Dov A Degen, Jyotsna Janardan, Katherine A Barraclough, Hans G Schneider, Thomas Barber, Howard Barton, Gregory Snell, Bronwyn Levvey, Rowan G Walker
BACKGROUND: There has been limited examination of the performance of glomerular filtration rate estimation (eGFR) equations in lung transplant populations. This study aimed to compare the performance of serum creatinine and cystatin C based eGFR equations with Tc-99m diethylenetriaminepentaacetic acid (DTPA) GFR measurements in individuals with end-stage lung disease, either prior to, or following, lung transplantation. METHODS: In this prospective observational study, participants underwent GFR measurements with Tc-99m Pentetate...
January 17, 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/28107795/adherence-to-therapies-in-cystic-fibrosis-a-targeted-literature-review
#3
Siva Narayanan, Jochen G Mainz, Smeet Gala, Harold Tabori, Daniel Grossoehme
Cystic fibrosis (CF) is a life-shortening condition with no cure. Available therapies relieving the symptoms of CF are complex and time-consuming. A comprehensive review assessing adherence to different CF therapies, association of adherence with outcomes, and factors influencing adherence could inform optimal patient management strategies. Areas covered: A targeted literature review of studies published from 2010-2016 assessed adherence to CF therapies. Nineteen studies qualified for inclusion. Adherence to CF therapies was sub-optimal, and varied by treatment, mode of treatment administration, age, season, time and method of adherence measurement...
January 20, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28107092/a-solid-pseudopapillary-neoplasm-of-the-pancreas-associated-with-igg4-related-pancreatitis
#4
Yuichiro Hatano, Keisuke Kawashima, Takuji Iwashita, Masaki Kimura, Masahito Shimizu, Akira Hara
A solid and cystic pancreatic lesion was incidentally found in a 62-year-old woman on abdominal computed tomography. The lesion was diagnosed as a solid pseudopapillary neoplasm by using endoscopic ultrasound-guided fine needle aspiration, and the tumor was resected. Histologically, the tumor cells had relatively small, round nuclei and papillary-like architecture in a hemorrhagic background. On immunohistochemical examination, the tumor cells were diffusely positive for nuclear β-catenin and cytoplasmic CD10...
November 1, 2016: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28106859/burkholderia-cepacia-complex-regulation-of-virulence-gene-expression-a-review
#5
REVIEW
Sílvia A Sousa, Joana R Feliciano, Tiago Pita, Soraia I Guerreiro, Jorge H Leitão
Burkholderia cepacia complex (Bcc) bacteria emerged as opportunistic pathogens in cystic fibrosis and immunocompromised patients. Their eradication is very difficult due to the high level of intrinsic resistance to clinically relevant antibiotics. Bcc bacteria have large and complex genomes, composed of two to four replicons, with variable numbers of insertion sequences. The complexity of Bcc genomes confers a high genomic plasticity to these bacteria, allowing their adaptation and survival to diverse habitats, including the human host...
January 19, 2017: Genes
https://www.readbyqxmd.com/read/28106773/determinants-of-serum-glycerophospholipid-fatty-acids-in-cystic-fibrosis
#6
Sławomira Drzymała-Czyż, Patrycja Krzyżanowska, Berthold Koletzko, Jan Nowak, Anna Miśkiewicz-Chotnicka, Jerzy A Moczko, Aleksandra Lisowska, Jarosław Walkowiak
The etiology of altered blood fatty acid (FA) composition in cystic fibrosis (CF) is understood only partially. We aimed to investigate the determinants of serum glycerophospholipids' FAs in CF with regard to the highest number of FAs and in the largest cohort to date. The study comprised 172 CF patients and 30 healthy subjects (HS). We assessed Fas' profile (gas chromatography/mass spectrometry), CF transmembrane conductance regulator (CFTR) genotype, spirometry, fecal elastase-1, body height and weight Z-scores, liver disease, diabetes and colonization by Pseudomonas aeruginosa...
January 18, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28103851/cystic-fibrosis-physicians-perspectives-on-the-timing-of-referral-for-lung-transplant-evaluation-a-survey-of-physicians-in-the-united-states
#7
Kathleen J Ramos, Ranjani Somayaji, Erika D Lease, Christopher H Goss, Moira L Aitken
BACKGROUND: Prior studies reveal that a significant proportion of patients with cystic fibrosis (CF) and advanced lung disease are not referred for lung transplant (LTx) evaluation. We sought to assess expert CF physician perspectives on the timing of LTx referral and investigate their LTx knowledge. METHODS: We developed an online anonymous survey that was distributed by the Cystic Fibrosis Foundation (CFF) to the medical directors of all CFF-accredited care centers in the United States in 2015...
January 19, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28102970/microbiomes-in-respiratory-health-and-disease-an-asia-pacific-perspective
#8
REVIEW
Sanjay H Chotirmall, Shaan L Gellatly, Kurtis F Budden, Micheál Mac Aogain, Shakti D Shukla, David L A Wood, Philip Hugenholtz, Kevin Pethe, Philip M Hansbro
There is currently enormous interest in studying the role of the microbiome in health and disease. Microbiome's role is increasingly being applied to respiratory diseases, in particular COPD, asthma, cystic fibrosis and bronchiectasis. The changes in respiratory microbiomes that occur in these diseases and how they are modified by environmental challenges such as cigarette smoke, air pollution and infection are being elucidated. There is also emerging evidence that gut microbiomes play a role in lung diseases through the modulation of systemic immune responses and can be modified by diet and antibiotic treatment...
February 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28102546/ataluren-and-similar-compounds-specific-therapies-for-premature-termination-codon-class-i-mutations-for-cystic-fibrosis
#9
REVIEW
Aisha A Aslam, Colin Higgins, Ian P Sinha, Kevin W Southern
BACKGROUND: Cystic fibrosis is a common life-shortening genetic disorder in the Caucasian population (less common in other ethnic groups) caused by the mutation of a single gene that codes for the production of the cystic fibrosis transmembrane conductance regulator protein. This protein coordinates the transport of salt (and bicarbonate) across cell surfaces and the mutation most notably affects the airways. In the lungs of people with cystic fibrosis, defective protein results in a dehydrated surface liquid and compromised mucociliary clearance...
January 19, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28102223/global-and-local-selection-acting-on-the-pathogen-stenotrophomonas-maltophilia-in-the-human-lung
#10
Hattie Chung, Tami D Lieberman, Sara O Vargas, Kelly B Flett, Alexander J McAdam, Gregory P Priebe, Roy Kishony
Bacterial populations diversify during infection into distinct subpopulations that coexist within the human body. Yet, it is unknown to what extent subpopulations adapt to location-specific selective pressures as they migrate and evolve across space. Here we identify bacterial genes under local and global selection by testing for spatial co-occurrence of adaptive mutations. We sequence 552 genomes of the pathogen Stenotrophomonas maltophilia across 23 sites of the lungs from a patient with cystic fibrosis. We show that although genetically close isolates colocalize in space, distant lineages with distinct phenotypes separated by adaptive mutations spread throughout the lung, suggesting global selective pressures...
January 19, 2017: Nature Communications
https://www.readbyqxmd.com/read/28100929/quantitative-computed-tomography-analysis-of-the-airways-in-patients-with-cystic-fibrosis-using-automated-software-correlation-with-spirometry-in-the-evaluation-of-severity
#11
Marcel Koenigkam Santos, Danilo Lemos Cruvinel, Marcelo Bezerra de Menezes, Sara Reis Teixeira, Elcio de Oliveira Vianna, Jorge Elias Júnior, José Antonio Baddini Martinez
OBJECTIVE: To perform a quantitative analysis of the airways using automated software, in computed tomography images of patients with cystic fibrosis, correlating the results with spirometric findings. MATERIALS AND METHODS: Thirty-four patients with cystic fibrosis were studied-20 males and 14 females; mean age 18 ± 9 years-divided into two groups according to the spirometry findings: group I (n = 21), without severe airflow obstruction (forced expiratory volume in first second [FEV1] > 50% predicted), and group II (n = 13), with severe obstruction (FEV1 ≤ 50% predicted)...
November 2016: Radiologia Brasileira
https://www.readbyqxmd.com/read/28100601/the-use-of-selective-fungal-culture-media-increases-detection-rates-of-fungi-in-the-cystic-fibrosis-respiratory-tract
#12
Gina Hong, Heather B Miller, Sarah Allgood, Richard Lee, Noah Lechtzin, Sean X Zhang
The prevalence of fungi in the respiratory tract of cystic fibrosis (CF) patients has risen. However, fungal surveillance is not routinely performed in most clinical centers in the United States, which may lead to an underestimation of the true prevalence of the problem. We conducted a prospective study comparing fungal detection rates for clinically important fungi (CIF) defined as Aspergillus species, Scedosporium species, Trichosporon species, and Exophiala dermatitidis in CF sputum using standard bacterial culture media and selective-fungal culture media, including Sabouraud dextrose agar with gentamicin (SDA), inhibitory mold agar (IMA), and brain-heart infusion agar (BHI) with chloramphenicol and gentamicin...
January 18, 2017: Journal of Clinical Microbiology
https://www.readbyqxmd.com/read/28100229/surveillance-of-life-long-antibiotics-a-review-of-antibiotic-prescribing-practices-in-an-australian-healthcare-network
#13
Jillian S Y Lau, Christopher Kiss, Erika Roberts, Kylie Horne, Tony M Korman, Ian Woolley
BACKGROUND: The rise of antimicrobial use in the twentieth century has significantly reduced morbidity due to infection, however it has also brought with it the rise of increasing resistance. Some patients are on prolonged, if not "life-long" course of antibiotics. The reasons for this are varied, and include non-infectious indications. We aimed to study the characteristics of this potential source of antibiotic resistance, by exploring the antibiotic dispensing practices and describing the population of patients on long-term antibiotic therapy...
January 18, 2017: Annals of Clinical Microbiology and Antimicrobials
https://www.readbyqxmd.com/read/28100217/variation-in-branchial-expression-among-insulin-like-growth-factor-binding-proteins-igfbps-during-atlantic-salmon-smoltification-and-seawater-exposure
#14
Jason P Breves, Chelsea K Fujimoto, Silas K Phipps-Costin, Ingibjörg E Einarsdottir, Björn Thrandur Björnsson, Stephen D McCormick
BACKGROUND: In preparation for migration from freshwater to marine habitats, Atlantic salmon (Salmo salar L.) undergo smoltification, a transformation that includes the acquisition of hyposmoregulatory capacity. The growth hormone (Gh)/insulin-like growth-factor (Igf) axis promotes the development of branchial ionoregulatory functions that underlie ion secretion. Igfs interact with a suite of Igf binding proteins (Igfbps) that modulate hormone activity. In Atlantic salmon smolts, igfbp4,-5a,-5b1,-5b2,-6b1 and-6b2 transcripts are highly expressed in gill...
January 18, 2017: BMC Physiology
https://www.readbyqxmd.com/read/28099811/nanomolar-potency-aminophenyl-1-3-5-triazine-activators-of-the-cystic-fibrosis-transmembrane-conductance-regulator-cftr-chloride-channel-for-pro-secretory-therapy-of-dry-eye-diseases
#15
Sujin Lee, Puay-Wah Phuan, Christian M Felix, Joseph-Anthony Tan, Marc H Levin, Alan S Verkman
Dry eye disorders are a significant health problem for which limited therapeutic options are available. CFTR is a major pro-secretory chloride channel at the ocular surface. We previously identified, by high-throughput screening, aminophenyl-1,3,5-triazine CFTRact-K089 (1) that activated CFTR with EC50 ~250 nM, which when delivered topically increased tear fluid secretion in mice and showed efficacy in an experimental dry eye model. Here, functional analysis of aminophenyl-1,3,5-triazine analogs elucidated structure-activity relationships for CFTR activation and identified substantially more potent analogs than 1...
January 18, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28099226/computed-tomography-in-cystic-fibrosis-combining-low-dose-techniques-and-iterative-reconstruction
#16
Johannes Kahn, David Kaul, Ulrich Grupp, Georg Böning, Diane Renz, Doris Staab, Vera Schreiter, Florian Streitparth
OBJECTIVE: The aim of the study was to evaluate the impact of iterative reconstruction (IR) and low-kilovolt technique on dose reduction and image quality of chest computed tomography scans obtained in patients with cystic fibrosis (CF). METHODS: A total of 124 CF patients were examined; group A (n = 63) was examined with a dose reduction protocol using IR and group B (n = 61) with a standard protocol using filtered back projection. Further subgroups with tube voltage modulations were established...
January 18, 2017: Journal of Computer Assisted Tomography
https://www.readbyqxmd.com/read/28097632/acquisition-and-adaptation-of-the-airway-microbiota-in-the-early-life-of-cystic-fibrosis-patients
#17
REVIEW
Sébastien Boutin, Alexander H Dalpke
Cystic fibrosis (CF) is a genetic disease in which bacterial infections of the airways play a major role in the long-term clinical outcome. In recent years, a number of next-generation sequencing (NGS)-based studies aimed at deciphering the structure and composition of the airways' microbiota. It was shown that the nasal cavity of CF patients displays dysbiosis early in life indicating a failure in the first establishment of a healthy microbiota. In contrast, within the conducting and lower airways, the establishment occurs normally first, but is sensitive to future dysbiosis including chronic infections with classical pathogens in later life...
December 2017: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/28096997/bronchial-tree-shaped-mucous-plug-in-cystic-fibrosis-imaging-guided-management
#18
Ignazio Salamone, Baldassare Mondello, Maria Cristina Lucanto, Simona Cristadoro, Mariangela Lombardo, Mario Barone
We report the case of a 17-year-old boy with cystic fibrosis (CF) who presented with persistent cough; after starting intravenous antibiotics for Pseudomonas aeruginosa he underwent a computed tomography (CT) scan of the chest. CT revealed extensive consolidation in the right lower lobe with relative bronchus obstruction; the cause of bronchial obstruction was detected in the mediastinal window, corresponding to a bronchial tree-shaped, thick, tenacious mucous plug. This was extracted 48 h after unresponsive bronchial washing and endobronchial instillation of rhDNAse, using foreign-body forceps, with subsequent resolution of cough...
March 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28096734/chest-ct-features-of-cystic-fibrosis-in-korea-comparison-with-non-cystic-fibrosis-diseases
#19
So Yeon Yang, Kyung Soo Lee, Min Jae Cha, Tae Jung Kim, Tae Sung Kim, Hyun Jung Yoon
OBJECTIVE: Cystic fibrosis (CF) is a rare congenital disease in Korea, and its clinical and imaging findings are unclear. The objective of our study was to describe the clinical and CT features of CF in Korea and compare its features with those of other diseases mimicking CF. MATERIALS AND METHODS: From November 1994 to December 2014, a presumptive diagnosis of CF was made in 23 patients based on clinical or radiological examination. After the exclusion of 10 patients without diagnostic confirmation, 13 patients were included in the study...
January 2017: Korean Journal of Radiology: Official Journal of the Korean Radiological Society
https://www.readbyqxmd.com/read/28096165/in-vitro-activity-of-ceftolozane-tazobactam-against-multidrug-resistant-non-fermenting-gram-negative-bacilli-isolated-from-patients-with-cystic-fibrosis
#20
Patrick Grohs, Gary Taieb, Philippe Morand, Iheb Kaibi, Isabelle Podglajen, Marie Lavollay, Jean-Luc Mainardi, Fabrice Compain
Ceftolozane-tazobactam was tested against 58 multidrug-resistant non-fermenting Gram-negative bacilli (35 Pseudomonas aeruginosa, 11 Achromobacter xylosoxydans and 12 Stenotrophomonas maltophilia) isolated from cystic fibrosis patients and compared to ceftolozane alone, ceftazidime, meropenem and piperacillin-tazobactam. Ceftolozane-tazobactam was the most active agent against P. aeruginosa but was inactive against A. xylosoxydans and S. maltophilia In time-kill experiments ceftolozane-tazobactam had complete bactericidal activity against 2/6 (33%) clinical isolates...
January 17, 2017: Antimicrobial Agents and Chemotherapy
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