keyword
MENU ▼
Read by QxMD icon Read
search

"Cystic fibrosis"

keyword
https://www.readbyqxmd.com/read/28441669/keep-them-breathing-cystic-fibrosis-pathophysiology-diagnosis-and-treatment
#1
Sheena D Brown, Rachel White, Phil Tobin
Cystic fibrosis (CF) affects more than 30,000 people in the United States and 80,000 people worldwide. This life-threatening genetic disorder causes a buildup of thick, viscous mucus secretions in various organ systems, most commonly the gastrointestinal, pulmonary, and genitourinary systems. This article reviews the clinical manifestations, diagnosis, and monitoring of patients with CF as well as guidelines for management and emerging pharmacologic treatments.
May 2017: JAAPA: Official Journal of the American Academy of Physician Assistants
https://www.readbyqxmd.com/read/28440913/piperacillin-tazobactam-versus-cefepime-incidence-of-acute-kidney-injury-in-combination-with-vancomycin-and-tobramycin-in-pediatric-cystic-fibrosis-patients
#2
Lisa K LeCleir, Rebecca S Pettit
BACKGROUND: Cystic fibrosis (CF) patients often receive prolonged courses of broad spectrum antibiotics, such as piperacillin-tazobactam or cefepime in combination with vancomycin and tobramycin. The objective of this study was to determine the difference in AKI for pediatric CF patients receiving piperacillin-tazobactam or cefepime in combination with vancomycin and tobramycin. METHODS: IRB approval from a single CF center was obtained for this retrospective cohort study...
April 25, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28440853/antibiotic-strategies-for-eradicating-pseudomonas-aeruginosa-in-people-with-cystic-fibrosis
#3
REVIEW
Simon C Langton Hewer, Alan R Smyth
BACKGROUND: Respiratory tract infection with Pseudomonas aeruginosa occurs in most people with cystic fibrosis. Once chronic infection is established, Pseudomonas aeruginosa is virtually impossible to eradicate and is associated with increased mortality and morbidity. Early infection may be easier to eradicate.This is an update of a Cochrane review first published in 2003, and previously updated in 2006, 2009 and 2014. OBJECTIVES: To determine whether antibiotic treatment of early Pseudomonas aeruginosa infection in children and adults with cystic fibrosis eradicates the organism, delays the onset of chronic infection, and results in clinical improvement...
April 25, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28440538/massive-nasal-polyposis-in-a-patient-with-newly-diagnosed-cystic-fibrosis
#4
Małgorzata Olszowiec-Chlebna, Krzysztof Trzciński, Iwona Stelmach
INTRODUCTION: Cystic fibrosis (CF) - is the most common fatal autosomal recessive disease in Caucasians. A number of reports have described patients who do not meet diagnostic criteria for cystic fibrosis. Atypical or nonclassic CF is characterised by normal or borderline sweat test, pancreatic sufficiency and a monosymptomatic phenotype. For these reasons clinicians should remain alert to the possibility of the occurrence of CF. CASE REPORT: We described a case presentation of massive nasal polyposis and recurrent sinusitis leading to the diagnosis ofcystic fibrosis in a 11-year-old male...
2017: Advances in Respiratory Medicine
https://www.readbyqxmd.com/read/28440535/socioeconomic-status-and-its-relationship-to-chronic-respiratory-disease
#5
Sonu Sahni, Ankoor Talwar, Sameer Khanijo, Arunabh Talwar
Socioeconomic status (SES) is defined as an individual's social or economic standing, and is a measure of an individual's or family's social or economic position or rank in a social group. It is a composite of several measures including income, education, occupation, location of residence or housing. Studies have found a lower SES has been linked to disproportionate access to health care in many diseases. There is emerging data in pulmonary diseases such as COPD, asthma, cystic fibrosis, pulmonary hypertension and other chronic respiratory conditions that allude to a similar observation noted in other chronic diseases...
2017: Advances in Respiratory Medicine
https://www.readbyqxmd.com/read/28440108/microbiology-of-the-upper-and-lower-airways-in-pediatric-cystic-fibrosis-patients
#6
Lindsay Sobin, Kosuke Kawai, Alexandria L Irace, Ozgul Gergin, Michael Cunningham, Gregory S Sawicki, Eelam A Adil
Objective To evaluate the microbiology of the upper and lower airways in pediatric cystic fibrosis (CF) patients who underwent sinus surgery. Study Design Retrospective case series with chart review. Setting Tertiary care children's hospital. Subjects and Methods A total of 201 paired sinus and pulmonary cultures from 105 CF patients were identified between 1996 and 2014. Demographics and culture results were analyzed. Results The mean age of patients was 11.2 ± 5.4 years (range, 1-27 years), and approximately one-half were female...
April 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28439630/myriocin-treatment-of-cf-lung-infection-and-inflammation-complex-analyses-for-enigmatic-lipids
#7
Anna Caretti, Michele Vasso, Fabiola Tecla Bonezzi, Andrea Gallina, Marco Trinchera, Alice Rossi, Raffaella Adami, Josefina Casas, Monica Falleni, Delfina Tosi, Alessandra Bragonzi, Riccardo Ghidoni, Cecilia Gelfi, Paola Signorelli
Our aim was to use quantitative and qualitative analyses to gain further insight into the role of ceramide in cystic fibrosis (CF). Sphingolipid ceramide is a known inflammatory mediator, and its accumulation in inflamed lung has been reported in different types of emphysema, chronic obstructive pulmonary disease and CF. CF is caused by a mutation of the chloride channel and associated with hyperinflammation of the respiratory airways and high susceptibility to ongoing infections. We have previously demonstrated that de novo ceramide synthesis is enhanced in lung inflammation and sustains Pseudomonas aeruginosa pulmonary infection in a CF murine model...
April 24, 2017: Naunyn-Schmiedeberg's Archives of Pharmacology
https://www.readbyqxmd.com/read/28439270/cytokine-regulation-of-na-k-cl-cotransporter-1-and-cystic-fibrosis-transmembrane-conductance-regulator-potential-role-in-pulmonary-inflammation-and-edema-formation
#8
REVIEW
Sarah Weidenfeld, Wolfgang M Kuebler
Pulmonary edema, a major complication of lung injury and inflammation, is defined as accumulation of extravascular fluid in the lungs leading to impaired diffusion of respiratory gases. Lung fluid balance across the alveolar epithelial barrier protects the distal airspace from excess fluid accumulation and is mainly regulated by active sodium transport and Cl(-) absorption. Increased hydrostatic pressure as seen in cardiogenic edema or increased vascular permeability as present in inflammatory lung diseases such as the acute respiratory distress syndrome (ARDS) causes a reversal of transepithelial fluid transport resulting in the formation of pulmonary edema...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28439239/development-of-automated-patch-clamp-technique-to-investigate-cftr-chloride-channel-function
#9
Arnaud Billet, Lionel Froux, John W Hanrahan, Frederic Becq
The chloride (Cl(-)) channel cystic fibrosis transmembrane conductance regulator (CFTR) is defective in cystic fibrosis (CF), and mutation of its encoding gene leads to various defects such as retention of the misfolded protein in the endoplasmic reticulum, reduced stability at the plasma membrane, abnormal channel gating with low open probability, and thermal instability, which leads to inactivation of the channel at physiological temperature. Pharmacotherapy is one major therapeutic approach in the CF field and needs sensible and fast tools to identify promising compounds...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28439038/pseudomonas-aeruginosa-derived-rhamnolipids-and-other-detergents-modulate-colony-morphotype-and-motility-in-the-burkholderia-cepacia-complex
#10
Steve P Bernier, Courtney Hum, Xiang Li, George A O'Toole, Nathan Magarvey, Michael G Surette
Competitive interactions mediated by released chemicals (e.g. toxins) are prominent in multispecies communities, but their effect at subinhibitory concentrations on susceptible bacteria is poorly understood. Although Pseudomonas aeruginosa and species of the Burkholderia cepacia complex (Bcc) can exist together as a co-infection in cystic fibrosis airways, P. aeruginosa toxins can kill Bcc species in vitro Consequently, these bacteria become an ideal in vitro model system to study the impact of sublethal levels of toxins on the biology of typical susceptible bacteria such as the Bcc when exposed to P...
April 24, 2017: Journal of Bacteriology
https://www.readbyqxmd.com/read/28438512/physical-performance-quality-of-life-and-sexual-satisfaction-evaluation-in-adults-with-cystic-fibrosis-an-unexplored-correlation
#11
K C A Aguiar, F A L Marson, C C S Gomez, M C Pereira, I A Paschoal, A F Ribeiro, J D Ribeiro
OBJECTIVE: Quality of life (QOL), sexual satisfaction (SS) and physical performance have been assessed in the management of numerous chronic diseases. METHODS: In this study, the following tests and surveys were applied: (i) QOL questionnaire [Cystic Fibrosis Questionnaire (CFQ)]; (ii) SS questionnaire (SSQ) [female sexual quotient (FSQ) and male sexual quotient (MSQ)]; (iii) 6-minute walk test (6MWT). Spearman's correlation was used for comparison between the data; the Mann-Whitney test was applied to analyze the difference between genders...
April 21, 2017: Revista Portuguesa de Pneumologia
https://www.readbyqxmd.com/read/28438500/ap2-%C3%AE-modulates-cystic-fibrosis-transmembrane-conductance-regulator-function-in-the-human-intestine
#12
Vandana Kumari, Shruti Desai, Nadia A Ameen
BACKGROUND: AP2 is a clathrin-based endocytic adaptor complex comprising α, β2, μ2 and σ2 subunits. μ2 regulates CFTR endocytosis. The α subunit interacts with CFTR in the intestine but its physiologic significance is unclear. METHODS: CFTR short circuit current was measured in intestinal T84 cells following shRNA knock down of AP2α (AP2αKD). Clathrin-coated structures (CCS) were immunolabeled and quantified in AP2αKD intestinal Caco2BBe (C2BBe) cells. GST tagged human AP2α appendage domain was cloned and its interaction with CFTR determined by GST pull down assay...
April 21, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28438499/rationalizing-endpoints-for-prospective-studies-of-pulmonary-exacerbation-treatment-response-in-cystic-fibrosis
#13
D R VanDevanter, S L Heltshe, J Spahr, V V Beckett, C L Daines, E C Dasenbrook, R L Gibson, R Jain, D B Sanders, C H Goss, P A Flume
BACKGROUND: Given the variability in pulmonary exacerbation (PEx) management within and between Cystic Fibrosis (CF) Care Centers, it is possible that some approaches may be superior to others. A challenge with comparing different PEx management approaches is lack of a community consensus with respect to treatment-response metrics. In this analysis, we assess the feasibility of using different response metrics in prospective randomized studies comparing PEx treatment protocols. METHODS: Response parameters were compiled from the recent STOP (Standardized Treatment of PEx) feasibility study...
April 21, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28437538/effects-of-diagnosis-by-newborn-screening-for-cystic-fibrosis-on-weight-and-length-in-the-first-year-of-life
#14
Daniel H Leung, Sonya L Heltshe, Drucy Borowitz, Daniel Gelfond, Margaret Kloster, James E Heubi, Michael Stalvey, Bonnie W Ramsey
Importance: Since the implementation of universal newborn screening (NBS) for cystic fibrosis (CF), the timing and magnitude of growth deficiency or its association with correlates of disease among infants with CF who underwent NBS has not been well described. Objective: To examine incremental weight gain, linear growth, and clinical features in the first year of life among infants with CF who underwent NBS. Design, Setting, and Participants: The Baby Observational and Nutrition Study (BONUS), a multicenter, longitudinal, observational cohort study, was conducted during regular CF clinic visits in the first 12 months of life at 28 US Cystic Fibrosis Foundation-accredited Care Centers from January 7, 2012, through May 31, 2015...
April 24, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28436621/risk-factors-for-mortality-before-age-18-years-in-cystic-fibrosis
#15
Susanna A McColley, Michael S Schechter, Wayne J Morgan, David J Pasta, Marcia L Craib, Michael W Konstan
BACKGROUND: Understanding early-life risk factors for childhood death in cystic fibrosis (CF) is important for clinical care, including the identification of effective interventions. METHODS: Data from the Epidemiologic Study of Cystic Fibrosis (ESCF) collected 1994-2005 were linked with the Cystic Fibrosis Foundation Patient Registry (CFFPR) demographic and mortality data from 2013. Inclusion criteria were ≥1 visit annually at age 3-5 years and ≥1 FEV1 measurement at age 6-8 years...
April 24, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28436419/nontuberculous-mycobacteria-in-gastrostomy-fed-patients-with-cystic-fibrosis
#16
H Al-Momani, A Perry, R Jones, S Bourke, S Doe, J Perry, A Anderson, T Forrest, I Forrest, M Griffin, M Brodlie, J Pearson, C Ward
Multi-drug resistant Mycobacterium abscessus complex (MABSC) is a form of Nontuberculous mycobacteria (NTM) of special, international concern in Cystic Fibrosis (CF). We hypothesised that gastric juice and percutaneous endoscopic gastrostomy (PEG) feeding devices might yield MABSC isolates. Gastric juice and sputa from sixteen adult PEG fed CF patients and five replaced PEG tubes were studied. Bacterial and fungal isolates were cultured. Mycobacterium were identified by rpoB, sodA and hsp65 gene sequencing and strain typed using variable number tandem repeat...
April 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28435675/microbiome-effects-on-immunity-health-and-disease-in-the-lung
#17
REVIEW
Shakti D Shukla, Kurtis F Budden, Rachael Neal, Philip M Hansbro
Chronic respiratory diseases, including asthma, chronic obstructive pulmonary disease (COPD) and cystic fibrosis (CF), are among the leading causes of mortality and morbidity worldwide. In the past decade, the interest in the role of microbiome in maintaining lung health and in respiratory diseases has grown exponentially. The advent of sophisticated multiomics techniques has enabled the identification and characterisation of microbiota and their roles in respiratory health and disease. Furthermore, associations between the microbiome of the lung and gut, as well as the immune cells and mediators that may link these two mucosal sites, appear to be important in the pathogenesis of lung conditions...
March 2017: Clinical & Translational Immunology
https://www.readbyqxmd.com/read/28435234/strengthening-care-teams-to-improve-adherence-in-cystic-fibrosis-a-qualitative-practice-assessment-and-quality-improvement-initiative
#18
Allison J Gardner, Alice L Gray, Staci Self, Jeffrey S Wagener
BACKGROUND: Treatment regimens for patients with cystic fibrosis (CF) are complex, time consuming, and burdensome, and adherence to CF treatment is suboptimal. CF care teams play a critical role in supporting patients' chronic self-management skills, but there is no uniform method for assessing patients' adherence to treatment or standard interventions to help patients improve when necessary. METHODS: Between May 2015 and March 2016, care team members from 10 CF centers in the USA participated in a practice assessment and quality improvement (QI) initiative...
2017: Patient Preference and Adherence
https://www.readbyqxmd.com/read/28434195/use-of-isavuconazole-in-a-patient-with-voriconazole-induced-qtc-prolongation
#19
Tracy P Trang, Alexandra M Hanretty, Charles Langelier, Katherine Yang
A 22-year-old woman with cystic fibrosis developed QTc-interval prolongation following lung transplantation in the setting of voriconazole therapy. After the discontinuation of voriconazole and initiation of isavuconazole, her QTc interval normalized. This case highlights the unique property of QTc interval shortening by isavuconazole among the triazole antifungals. This article is protected by copyright. All rights reserved.
April 23, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28433659/carbonic-anhydrase-xii-functions-in-health-and-disease
#20
REVIEW
Abdul Waheed, William S Sly
Human CAXII was initially identified as a cancer marker in different cancers and tumors. Expression of CAXII is regulated by hypoxia and estrogen receptors. CAXII expression has been also detected in several tissues, whereas in cancer and tumor tissues its expression is several fold higher. In brain tumors, an alternatively spliced form of CAXII is expressed. Higher expression of CAXII in breast cancer is indicative of lower grade disease. CAXII plays a key role in several physiological functions. Mutation in the CAXII gene causes cystic fibrosis-like syndrome and salt wasting disease...
April 19, 2017: Gene
keyword
keyword
15256
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"