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https://www.readbyqxmd.com/read/28449285/research-review-childhood-chronic-physical-illness-and-adult-emotional-health-a-systematic-review-and-meta-analysis
#1
REVIEW
Ekin Secinti, Ellen J Thompson, Marcus Richards, Darya Gaysina
BACKGROUND: Childhood chronic physical illness is associated with a greater vulnerability for emotional problems (i.e. depression and anxiety) in childhood. However, little is known about life-long effects of childhood chronic physical illness on mental health. The present study aims to systematically review evidence for associations between eight chronic physical illnesses with childhood onset (arthritis, asthma, cancer, chronic renal failure, congenital heart disease, cystic fibrosis, type 1 diabetes, and epilepsy) and adult emotional problems...
April 27, 2017: Journal of Child Psychology and Psychiatry, and Allied Disciplines
https://www.readbyqxmd.com/read/28448979/combination-of-correctors-rescues-cftr-transmembrane-domain-mutants-by-mitigating-their-interactions-with-proteostasis
#2
Miquéias Lopes-Pacheco, Clément Boinot, Inna Sabirzhanova, Daniele Rapino, Liudmila Cebotaru
BACKGROUND/AIMS: Premature degradation of mutated cystic fibrosis transmembrane conductance regulator (CFTR) protein causes cystic fibrosis (CF), the commonest Mendelian disease in Caucasians. Despite recent advances in precision medicines for CF patients, many CFTR mutants have not been characterized and the effects of these new therapeutic approaches are still unclear for those mutants. METHODS: Cells transfected or stably expressing four CFTR transmembrane-domain mutants (G85E, E92K, L1077P, and M1101K) were used to: 1) characterize the mutants according to their protein expression, thermal sensitivity, and degradation pathways; 2) evaluate the effects of correctors in rescuing them; and 3) explore the effects of correctors on CFTR interactions with proteostasis components...
April 25, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28448633/a-commensal-streptococcus-hijacks-a-pseudomonas-aeruginosa-exopolysaccharide-to-promote-biofilm-formation
#3
Jessica A Scoffield, Dingyu Duan, Fan Zhu, Hui Wu
Pseudomonas aeruginosa causes devastating chronic pulmonary infections in cystic fibrosis (CF) patients. Although the CF airway is inhabited by diverse species of microorganisms interlaced within a biofilm, many studies focus on the sole contribution of P. aeruginosa pathogenesis in CF morbidity. More recently, oral commensal streptococci have been identified as cohabitants of the CF lung, but few studies have explored the role these bacteria play within the CF biofilm. We examined the interaction between P...
April 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/28447619/little-cigars-are-more-toxic-than-cigarettes-and-uniquely-change-the-airway-gene-and-protein-expression
#4
Arunava Ghosh, Sabri H Abdelwahab, Steven L Reeber, Boris Reidel, Abigail J Marklew, Andrew J Garrison, Shernita Lee, Hong Dang, Amy H Herring, Gary L Glish, Mehmet Kesimer, Robert Tarran
Little cigars (LCs) are regulated differently than cigarettes, allowing them to be potentially targeted at youth/young adults. We exposed human bronchial epithelial cultures (HBECs) to air or whole tobacco smoke from cigarettes vs. LCs. Chronic smoke exposure increased the number of dead cells, lactate dehydrogenase release, and interleukin-8 (IL-8) secretion and decreased apical cilia, cystic fibrosis transmembrane conductance regulator (CFTR) protein levels, and transepithelial resistance. These adverse effects were significantly greater in LC-exposed HBECs than cigarette exposed cultures...
April 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28447578/proprotein-convertase-subtilisin-kexin-type-9-affects-insulin-but-not-lipid-metabolism-in-cystic-fibrosis
#5
Adèle Coriati, Elizabeth Arslanian, Guillaume F Bouvet, Annik Prat, Nabil G Seidah, Rémi Rabasa-Lhoret, Yves Berthiaume
PURPOSE: Cystic Fibrosis (CF) is the most common genetic disorder and, with improved survival, glucose abnormalities have emerged as a major comorbidity. Proprotein convertase subtilisin/kexin type 9 (PCSK9), a regulator of plasma LDL-cholesterol homeostasis, is associated with lipid and glucose metabolism in healthy individuals. Here we report on the link between PCSK9 and markers of metabolism in CF. METHODS: Cross-sectional analysis was performed on CF patients (≥ 18 years, N=94) from the Montreal Cohort, without known diabetes, and on healthy individuals (N=19)...
April 26, 2017: Clinical and Investigative Medicine. Médecine Clinique et Experimentale
https://www.readbyqxmd.com/read/28447292/ecology-of-the-human-opportunistic-black-yeast-exophiala-dermatitidis-indicates-preference-for-human-made-habitats
#6
Monika Novak Babič, Jerneja Zupančič, Nina Gunde-Cimerman, Sybren de Hoog, Polona Zalar
Exophiala dermatitidis is an ascomycetous black yeast from the order Chaetothyriales. Its growth characteristics include the polymorphic life cycle, ability to grow at high and low temperatures, at a wide pH range, survival at high concentrations of NaCl, and survival at high UV and radioactive radiation. Exophiala dermatitidis causes deep or localized phaeohyphomycosis in immuno-compromised people worldwide and is regularly encountered in the lungs of cystic fibrosis patients. Regardless of numerous ecological studies worldwide, little is known about its natural habitat or the possible infection routes...
April 26, 2017: Mycopathologia
https://www.readbyqxmd.com/read/28446577/population-dynamics-of-staphylococcus-aureus-in-cystic-fibrosis-patients-to-determine-transmission-events-utilizing-wgs
#7
Andrea Ankrum, Barry G Hall
Strict infection control practices have been implemented for healthcare visits by Cystic Fibrosis patients in an attempt to prevent transmission of important pathogens. This study used whole genome sequencing (WGS) to determine strain relatedness and assess population dynamics of Staphylococcus aureus isolates from a cohort of CF patients as assessed by strain relatedness. 311 S. aureus isolates were collected from respiratory cultures of 115 CF patients during a 22 month study period. Whole genome sequencing was performed and using SNP analysis, phylogenetic trees were assembled to determine relatedness between isolates...
April 26, 2017: Journal of Clinical Microbiology
https://www.readbyqxmd.com/read/28446570/prevalence-and-outcomes-of-achromobacter-species-infections-in-adults-with-cystic-fibrosis-a-north-american-cohort-study
#8
B D Edwards, J Greysson-Wong, R Somayaji, B Waddell, F J Whelan, D G Storey, H R Rabin, M G Surette, M D Parkins
Introduction:Achromobacter species are now increasingly recognized in CF, with unclear epidemiology and impact.Methods: We studied a cohort of patients attending a Canadian adult CF clinic who had positive sputum cultures for Achromobacter species from 1984-2013. Infection was categorized as transient or persistent (≥50% positive cultures in one year). Those with persistent infection were matched 2:1 with age, sex, and time-matched controls without history of Achromobacter infection and mixed effects models used to assess pulmonary exacerbation(PEx) frequency and lung function decline...
April 26, 2017: Journal of Clinical Microbiology
https://www.readbyqxmd.com/read/28446564/lung-lymphoid-neogenesis-in-cystic-fibrosis-a-model-of-adaptive-responses%C3%A2-to%C3%A2-bacteria
#9
EDITORIAL
Sophie T Gohy, Maha Z Ladjemi, Charles Pilette
No abstract text is available yet for this article.
April 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28446558/pseudomonas-aeruginosa-adaptation-and-diversification-in-the-non-cystic-fibrosis-bronchiectasis-lung
#10
Yasmin Hilliam, Matthew P Moore, Iain L Lamont, Diana Bilton, Charles S Haworth, Juliet Foweraker, Martin J Walshaw, David Williams, Joanne L Fothergill, Anthony De Soyza, Craig Winstanley
To characterise Pseudomonas aeruginosa populations during chronic lung infections of non-cystic fibrosis bronchiectasis patients, we used whole-genome sequencing to 1) assess the diversity of P. aeruginosa and the prevalence of multilineage infections; 2) seek evidence for cross-infection or common source acquisition; and 3) characterise P. aeruginosa adaptations.189 isolates, obtained from the sputa of 91 patients attending 16 adult bronchiectasis centres in the UK, were whole-genome sequenced.Bronchiectasis isolates were representative of the wider P...
April 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28446556/bacteria-driven-peribronchial-lymphoid-neogenesis-in-bronchiectasis-and-cystic-fibrosis
#11
Justine Frija-Masson, Clémence Martin, Lucile Regard, Marie-Noëlle Lothe, Lhousseine Touqui, Aurélie Durand, Bruno Lucas, Diane Damotte, Marco Alifano, Isabelle Fajac, Pierre-Régis Burgel
We aimed to characterise lymphoid neogenesis in bronchiectasis and cystic fibrosis (CF) lungs and to examine the role of bacterial infection.Lymphoid aggregates were examined using immunohistochemical staining and morphometric analysis in surgical lung sections obtained from nonsmokers and patients with bronchiectasis or CF. Sterile, Pseudomonas aeruginosa- or Staphylococcus aureus-coated agarose beads were instilled intratracheally in mice. Kinetics of lymphoid neogenesis and chemokine expression were examined over 14 days...
April 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28446387/risk-factors-for-persistent-methicillin-resistant-staphylococcus-aureus-infection-in-cystic-fibrosis
#12
Mark T Jennings, Elliot C Dasenbrook, Noah Lechtzin, Michael P Boyle, Christian A Merlo
BACKGROUND: Methicillin-resistant Staphylococcus aureus (MRSA) has emerged as an important pathogen in cystic fibrosis (CF). Over 25% of individuals in the United States with CF are found to have MRSA in respiratory culture specimens, and persistent MRSA infection has been associated with more rapid decline in lung function and increased mortality. The objective of this study was to investigate clinical and demographic characteristics that are associated with the development of persistent MRSA infection in a CF population...
April 23, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28445932/high-cftr-expression-in-philadelphia-chromosome-positive-acute-leukemia-protects-and-maintains-continuous-activation-of-bcr-abl-and-related-signaling-pathways-in-combination-with-pp2a
#13
Xi Yang, Tianyou Yan, Yuping Gong, Xuehua Liu, Huaqin Sun, Wenming Xu, Chunsen Wang, Duolan Naren, Yuhuan Zheng
Cystic fibrosis transmembrane conductance regulator (CFTR) is classified as an anion channel transporter of Cl- and HCO3-. Through interactions with its PDZ domain, CFTR is capable of regulating other proteins, such as protein phosphatase 2A (PP2A). The aberrant expression and mutation of CFTR have been observed in several tumor, but not in philadelphia chromosome-positive(Ph+) acute leukemia, including Ph+ B cell acute lymphoblastic leukemia(Ph+ B-ALL) and chronic myelogenous leukemia blast crisis phases (CML-BC)...
April 11, 2017: Oncotarget
https://www.readbyqxmd.com/read/28445890/non-cystic-fibrosis-bronchiectasis-from-programmatic-management-to-personalized-medicine
#14
Oxana Munteanu, Helmut J F Salzer
No abstract text is available yet for this article.
April 27, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28445004/cftr-dependent-chloride-efflux-in-cystic-fibrosis-mononuclear-cells-is-increased-by-ivacaftor-therapy
#15
Lorenzo Guerra, Susanna D'Oria, Maria Favia, Stefano Castellani, Teresa Santostasi, Angela M Polizzi, Maria A Mariggiò, Crescenzio Gallo, Valeria Casavola, Pasqualina Montemurro, Giuseppina Leonetti, Antonio Manca, Massimo Conese
AIM: The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) potentiator ivacaftor (Kalydeco®) improves clinical outcome in G551D cystic fibrosis (CF) patients. Here, we have investigated whether ivacaftor has a clinical impact on non-G551D gating mutations and function of circulating leukocytes as well. METHODS: Seven patients were treated with ivacaftor and evaluated at baseline, and at 1-3 and 6 months. Besides clinical and systemic inflammatory parameters, circulating mononuclear cells (MNC) were evaluated for CFTR-dependent chloride efflux by spectrofluorimetry, neutrophils for oxidative burst by cytofluorimetry and HVCN1 mRNA expression by real time PCR...
April 26, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28444650/-newborn-screening-on-cystic-fibrosis-in-germany-comparison-of-the-new-screening-protocol-with-an-alternative-protocol
#16
Olaf Sommerburg, Mirjam Stahl, Jutta Hammermann, Jürgen G Okun, Andreas Kulozik, Georg Hoffmann, Marcus Mall
Background For the new cystic fibrosis (CF) newborn screening program in Germany the Federal Joint Committee (G-BA) implemented a new screening protocol using immunoreactive trypsinogen (IRT) as first and pancreatitis associated protein (PAP) as second tier. Gene analysis with a panel of 31 CFTR-mutations is used as third tier to increase the positive predictive value (PPV) which is known to be low in pure biochemical IRT/PAP protocols. Methods For post hoc analysis the data pool (n=372 906) of a study evaluating a pure biochemical IRT/PAP protocol was used for assessment of the 3-step G-BA protocol in comparison with an alternative screening protocol recommended by the authors...
March 2017: Klinische Pädiatrie
https://www.readbyqxmd.com/read/28444649/-cystic-fibrosis-screening-in-germany
#17
L Gortner
No abstract text is available yet for this article.
March 2017: Klinische Pädiatrie
https://www.readbyqxmd.com/read/28443679/emerging-roles-of-carbonyl-sulfide-cos-in-chemical-biology-sulfide-transporter-or-gasotransmitter
#18
Andrea K Steiger, Yu Zhao, Michael D Pluth
SIGNIFICANCE: Carbonyl sulfide (COS) is the most prevalent sulfur-containing gas in Earth's atmosphere and plays important roles in the global sulfur cycle. COS has been implicated in origin of life peptide ligation, is the primary energy source for certain bacteria, and has been detected in mammalian systems. Despite this long and intertwined history with terrestrial biology, limited attention has focused on potential roles of COS as a biological mediator. Recent Advances. Although bacterial COS production is well documented, definitive sources of mammalian COS production have not been confirmed...
April 26, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/28443234/plastic-bronchitis-an-unusual-complication-of-acute-chest-syndrome-in-adult
#19
Sarah Feray, Pierre Mora, Maxens Decavele, Tài Pham, El Mahdi Hafiani, Muriel Fartoukh
Plastic bronchitis is used to designate endobronchial plugs of rubber-like consistency that form into bronchial trees. It has been described in several diseases like asthma, cystic fibrosis, pulmonary infection, cyanotic congenital heart disease and in few young children with homozygous sickle cell disease. We report the first sickle cell adult case of plastic bronchitis during acute chest syndrome. He developed severe acute respiratory distress syndrome. This unusual presentation related to obstruction by voluminous casts may alert physicians to focus more on the bronchi in sickle cell patients...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28442689/wound-healing-and-glaucoma-surgery-modulating-the-scarring-process-with-conventional-antimetabolites-and-new-molecules
#20
Gábor Holló
Subconjunctival absorption of aqueous humor is an essential part of glaucoma filtration surgery. Mitomycin C (MMC) and 5-fluorouracil have been used to reduce postoperative episcleral fibrosis and scar formation in the filtering bleb area for more than 2 decades. Both antimetabolites have also been frequently injected before needling revision of failing filtering blebs. Recently, MMC was also tried in tube surgery and nonpenetrating filtering surgery, but its usefulness in these applications has not yet been determined...
2017: Developments in Ophthalmology
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