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https://www.readbyqxmd.com/read/29769279/microbial-interactions-in-the-cystic-fibrosis-airway
#1
Ann M Granchelli, Frederick R Adler, Ruth H Keogh, Christiana Kartsonaki, David R Cox, Theodore G Liou
Interactions in the airway ecology of cystic fibrosis may alter organism persistence and clinical outcomes. Better understanding of such interactions could guide clinical decisions. We fitted logistic regression models using generalized estimating equations to longitudinal two-year patient cohorts in the Cystic Fibrosis Foundation Patient Registry, 2003-2011 to study associations between airway organisms present in each calendar year and their presence in the subsequent year. Models were adjusted for clinical characteristics and multiple observations per patient and tested for sensitivity to cystic fibrosis-specific treatments...
May 16, 2018: Journal of Clinical Microbiology
https://www.readbyqxmd.com/read/29764481/a-total-pleural-covering-of-absorbable-cellulose-mesh-prevents-pneumothorax-recurrence-in-patients-with-birt-hogg-dub%C3%A3-syndrome
#2
Teruaki Mizobuchi, Masatoshi Kurihara, Hiroki Ebana, Sumitaka Yamanaka, Hideyuki Kataoka, Shouichi Okamoto, Etsuko Kobayashi, Toshio Kumasaka, Kuniaki Seyama
BACKGROUND: Birt-Hogg-Dubé syndrome (BHDS) is a recently recognized inherited multiple cystic lung disease causing recurrent pneumothoraces. Similarly to the lesions in patients with lymphangioleiomyomatosis (LAM), the pulmonary cysts are innumerable and widely dispersed and cannot all be removed. We recently described a total pleural covering (TPC) that covers the entire visceral pleura with oxidized regenerated cellulose (ORC) mesh. TPC successfully prevented the recurrence of pneumothorax in LAM patients...
May 15, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29741792/frontline-science-pathological-conditioning-of-human-neutrophils-recruited-to-the-airway-milieu-in-cystic-fibrosis
#3
Osric A Forrest, Sarah A Ingersoll, Marcela K Preininger, Julie Laval, Dominique H Limoli, Milton R Brown, Frances E Lee, Brahmchetna Bedi, Ruxana T Sadikot, Joanna B Goldberg, Vin Tangpricha, Amit Gaggar, Rabindra Tirouvanziam
RATIONALE: Recruitment of neutrophils to the airways, and their pathological conditioning therein, drive tissue damage and coincide with the loss of lung function in patients with cystic fibrosis (CF). So far, these key processes have not been adequately recapitulated in models, hampering drug development. Here, we hypothesized that the migration of naïve blood neutrophils into CF airway fluid in vitro would induce similar functional adaptation to that observed in vivo, and provide a model to identify new therapies...
May 9, 2018: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/29733727/precision-medicine-in-action-the-impact-of-ivacaftor-on-cystic-fibrosis-related-hospitalizations
#4
Lisa B Feng, Scott D Grosse, Ridgely Fisk Green, Aliza K Fink, Gregory S Sawicki
Cystic fibrosis is a life-threatening genetic disease that causes severe damage to the lungs. Ivacaftor, the first drug that targeted the underlying defect of the disease caused by specific mutations, is a sterling example of the potential of precision medicine. Clinical trial and registry studies showed that ivacaftor improved outcomes and reduced hospitalizations. Our study used US administrative claims data to assess the real-world effectiveness of ivacaftor. Comparing twelve-month rates before and after starting the use of ivacaftor among people who initiated therapy during 2012-2015, we found that overall and cystic fibrosis-related inpatient admissions fell by 55 percent and 81 percent, respectively...
May 2018: Health Affairs
https://www.readbyqxmd.com/read/29715700/lung-ultrasound-findings-in-congenital-pulmonary-airway-malformation
#5
Nadya Yousef, Mostafa Mokhtari, Philippe Durand, Francesco Raimondi, Fiorella Migliaro, Alexandra Letourneau, Pierre Tissières, Daniele De Luca
BACKGROUND AND OBJECTIVE:  Congenital pulmonary airway malformation (CPAM) is a group of rare congenital malformations of the lung and airways. Lung ultrasound (LU) is increasingly used to diagnose neonatal respiratory diseases since it is quick, easy to learn, and radiation-free, but no formal data exist for congenital lung malformations. We aimed to describe LU findings in CPAM neonates needing neonatal intensive care unit (NICU) admission and to compare them with a control population...
May 1, 2018: American Journal of Perinatology
https://www.readbyqxmd.com/read/29707562/the-clinical-utility-of-lung-clearance-index-in-early-cystic-fibrosis-lung-disease-is-not-impacted-by-the-number-of-multiple-breath-washout-trials
#6
Rachel E Foong, Alana J Harper, Billy Skoric, Louise King, Lidija Turkovic, Miriam Davis, Charles C Clem, Tim Rosenow, Stephanie D Davis, Sarath Ranganathan, Graham L Hall, Kathryn A Ramsey
The lung clearance index (LCI) from the multiple-breath washout (MBW) test is a promising surveillance tool for pre-school children with cystic fibrosis (CF). Current guidelines for MBW testing recommend that three acceptable trials are required. However, success rates to achieve these criteria are low in children aged <7 years and feasibility may improve with modified pre-school criteria that accepts tests with two acceptable trials. This study aimed to determine if relationships between LCI and clinical outcomes of CF lung disease differ when only two acceptable MBW trials are assessed...
January 2018: ERJ Open Research
https://www.readbyqxmd.com/read/29680149/recent-progress-in-the-discovery-and-development-of-small-molecule-modulators-of-cftr
#7
Phil R Kym, Xueqing Wang, Mathieu Pizzonero, Steven E Van der Plas
Cystic fibrosis (CF) is a genetic disorder driven by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. While different mutations lead to varying levels of disease severity, the most common CFTR F508del mutation leads to defects in protein stability, trafficking to the cell membrane and gating of chloride ions. Recently, advances in medicinal chemistry have led to the identification small-molecule drugs that result in significant clinical efficacy in improving lung function in CF patients...
2018: Progress in Medicinal Chemistry
https://www.readbyqxmd.com/read/29676646/ambient-air-pollution-and-clinical-implications-for-susceptible-populations
#8
Laura G Hooper, Joel D Kaufman
Air pollution is associated with a diversity of health effects, and evidence for a causal relationship with specific diseases exists. Exposure to air pollution is ubiquitous and typically beyond the control of the individual; the resulting health burden for the population can be high. Disproportionate effects are seen in individuals who have increased susceptibility to air pollution owing to individual- or community-level characteristics. As studies grow increasingly sophisticated, the understanding of who comprises the susceptible population continuously expands...
April 2018: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29593867/joint-hierarchical-gaussian-process-model-with-application-to-personalized-prediction-in-medical-monitoring
#9
Leo L Duan, Xia Wang, John P Clancy, Rhonda D Szczesniak
A two-level Gaussian process (GP) joint model is proposed to improve personalized prediction of medical monitoring data. The proposed model is applied to jointly analyze multiple longitudinal biomedical outcomes, including continuous measurements and binary outcomes, to achieve better prediction in disease progression. At the population level of the hierarchy, two independent GPs are used to capture the nonlinear trends in both the continuous biomedical marker and the binary outcome, respectively; at the individual level, a third GP, which is shared by the longitudinal measurement model and the longitudinal binary model, induces the correlation between these two model components and strengthens information borrowing across individuals...
2018: Stat: the ISI's Journal for Rapid Dissemination of Statistics Research
https://www.readbyqxmd.com/read/29543980/macrolide-antibiotics-for-bronchiectasis
#10
REVIEW
Carol Kelly, James D Chalmers, Iain Crossingham, Nicola Relph, Lambert M Felix, David J Evans, Stephen J Milan, Sally Spencer
BACKGROUND: Bronchiectasis is a chronic respiratory disease characterised by abnormal and irreversible dilatation and distortion of the smaller airways. Bacterial colonisation of the damaged airways leads to chronic cough and sputum production, often with breathlessness and further structural damage to the airways. Long-term macrolide antibiotic therapy may suppress bacterial infection and reduce inflammation, leading to fewer exacerbations, fewer symptoms, improved lung function, and improved quality of life...
March 15, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29540993/the-egfr-adam17-axis-in-chronic-obstructive-pulmonary-disease-and-cystic-fibrosis-lung-pathology
#11
REVIEW
Marta Stolarczyk, Bob J Scholte
Chronic obstructive pulmonary disease (COPD) and cystic fibrosis (CF) share molecular mechanisms that cause the pathological symptoms they have in common. Here, we review evidence suggesting that hyperactivity of the EGFR/ADAM17 axis plays a role in the development of chronic lung disease in both CF and COPD. The ubiquitous transmembrane protease A disintegrin and metalloprotease 17 (ADAM17) forms a functional unit with the EGF receptor (EGFR), in a feedback loop interaction labeled the ADAM17/EGFR axis. In airway epithelial cells, ADAM17 sheds multiple soluble signaling proteins by proteolysis, including EGFR ligands such as amphiregulin (AREG), and proinflammatory mediators such as the interleukin 6 coreceptor (IL-6R)...
2018: Mediators of Inflammation
https://www.readbyqxmd.com/read/29461800/antisense-inhibitors-retain-activity-in-pulmonary-models-of-burkholderia-infection
#12
Seth M Daly, Carolyn R Sturge, Kimberly R Marshall-Batty, Christina F Felder-Scott, Raksha Jain, Bruce L Geller, David E Greenberg
The Burkholderia cepacia complex is a group of Gram-negative bacteria that are opportunistic pathogens in immunocompromised individuals, such as those with cystic fibrosis (CF) or chronic granulomatous disease (CGD). Burkholderia are intrinsically resistant to many antibiotics and the lack of antibiotic development necessitates novel therapeutics. Peptide-conjugated phosphorodiamidate morpholino oligomers are antisense molecules that inhibit bacterial mRNA translation. Targeting of PPMOs to the gene acpP, which is essential for membrane synthesis, lead to defects in the membrane and ultimately bactericidal activity...
March 1, 2018: ACS Infectious Diseases
https://www.readbyqxmd.com/read/29274943/the-association-between-staphylococcus-aureus-and-subsequent-bronchiectasis-in-children-with-cystic-fibrosis
#13
Daan Caudri, Lidija Turkovic, Jolyn Ng, Nicholas H de Klerk, Tim Rosenow, Graham L Hall, Sarath C Ranganathan, Peter D Sly, Stephen M Stick
BACKGROUND: Staphylococcus aureus (S. aureus) may be related to more rapid progression of cystic fibrosis (CF) lung disease. METHODS: In the AREST CF cohort study, children diagnosed with CF undergo annual bronchoscopies with bronchoalveolar lavage and ultra-low-dose, chest computed tomography (CT) up to 6-years-old. Spirometry was assessed 3-monthly from the age of 4years. Associations between de novo S. aureus acquisition before school age and CT and lung function at ages 5-7years were investigated...
December 21, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29247372/smoking-related-lung-abnormalities-on-computed-tomography-images-comparison-with-pathological-findings
#14
REVIEW
Tae Iwasawa, Tamiko Takemura, Takashi Ogura
Smoking-related lung abnormalities are now an increasing public health concern. According to the findings of large-cohort studies, approximately 8% of smokers have interstitial lung abnormalities, which are associated with a relatively high risk of all-cause mortality. We reviewed the radiological and pathological findings of smoking-related interstitial lung diseases, such as respiratory bronchiolitis-interstitial lung disease, desquamative interstitial pneumonia, and airspace enlargement with fibrosis. We have also discussed the histological basis of unclassifiable interstitial pneumonia in smokers, which exhibits airway-centered cystic lesions with fibrosis...
March 2018: Japanese Journal of Radiology
https://www.readbyqxmd.com/read/29227218/association-between-sequevar-and-antibiotic-treatment-outcome-in-patients-with-mycobacterium-abscessus-complex-infections-in-japan
#15
Shiomi Yoshida, Kazunari Tsuyuguchi, Takehiko Kobayashi, Motohisa Tomita, Yoshikazu Inoue, Seiji Hayashi, Katsuhiro Suzuki
PURPOSE: Macrolide susceptibility differs between subspecies in the Mycobacterium abscessus complex, likely due to differences in erm(41) sequevars. Patients with M. abscessus complex infection generally show poor clinical outcomes in response to antibiotic treatment. Here, the association between genotype and treatment outcome was investigated. METHODOLOGY: We collected 69 isolates from 35 patients with non-cystic fibrosis bronchiectasis: 24 had M. abscessus complex lung disease and non-cystic fibrosis bronchiectasis, and 11 were colonized...
January 2018: Journal of Medical Microbiology
https://www.readbyqxmd.com/read/29217601/novel-magnetic-resonance-technique-for-functional-imaging-of-cystic-fibrosis-lung-disease
#16
Sylvia Nyilas, Grzegorz Bauman, Gregor Sommer, Enno Stranzinger, Orso Pusterla, Urs Frey, Insa Korten, Florian Singer, Carmen Casaulta, Oliver Bieri, Philipp Latzin
Lung function tests are commonly used to monitor lung disease in cystic fibrosis (CF). While practical, they cannot locate the exact origin of functional impairment. Contemporary magnetic resonance imaging (MRI) techniques provide information on the location of disease but the need for contrast agents constrains their repeated application. We examined the correlation between functional MRI, performed without administration of contrast agent, and lung clearance index (LCI) from nitrogen multiple-breath washout (N2 -MBW)...
December 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29190650/reduced-bone-length-growth-plate-thickness-bone-content-and-igf-i-as-a-model-for-poor-growth-in-the-cftr-deficient-rat
#17
Michael S Stalvey, Viktoria Havasi, Katherine L Tuggle, Dezhi Wang, Susan Birket, Steve M Rowe, Eric J Sorscher
BACKGROUND: Reduced growth and osteopenia are common in individuals with cystic fibrosis (CF). Additionally, improved weight and height are associated with better lung function and overall health in the disease. Mechanisms for this reduction in growth are not understood. We utilized a new CFTR knockout rat to evaluate growth in young CF animals, via femur length, microarchitecture of bone and growth plate, as well as serum IGF-I concentrations. METHODS: Femur length was measured in wild-type (WT) and SD-CFTRtm1sage (Cftr-/-) rats, as a surrogate marker for growth...
2017: PloS One
https://www.readbyqxmd.com/read/29176899/supine-posture-changes-lung-volumes-and-increases-ventilation-heterogeneity-in-cystic-fibrosis
#18
Laurie J Smith, Kenneth A Macleod, Guilhem J Collier, Felix C Horn, Helen Sheridan, Ina Aldag, Chris J Taylor, Steve Cunningham, Jim M Wild, Alex Horsley
INTRODUCTION: Lung Clearance Index (LCI) is recognised as an early marker of cystic fibrosis (CF) lung disease. The effect of posture on LCI however is important when considering longitudinal measurements from infancy and when comparing LCI to imaging studies. METHODS: 35 children with CF and 28 healthy controls (HC) were assessed. Multiple breath washout (MBW) was performed both sitting and supine in triplicate and analysed for LCI, Scond, Sacin, and lung volumes...
2017: PloS One
https://www.readbyqxmd.com/read/29169151/cystic-lung-disease-in-genetic-syndromes-with-deficient-tumor-suppressor-gene-function
#19
Cécile Daccord, Laurent P Nicod, Romain Lazor
Cystic lung diseases constitute a distinct group of rare lung disorders, among which two result from monogenic defects affecting tumor suppressor genes: lymphangioleiomyomatosis, either sporadic or associated with tuberous sclerosis complex, and Birt-Hogg-Dubé syndrome. These disorders have similarities in their clinical expression, including occurrence in young adults, multiple pulmonary cysts, recurrent pneumothorax, skin hamartomas, and renal tumors. However, they markedly differ in their gender distribution, pathogenesis, disease course, and prognosis...
November 23, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/29163413/lipoprotein-glycosylation-by-protein-o-mannosyltransferase-mab_1122c-contributes-to-low-cell-envelope-permeability-and-antibiotic-resistance-of-mycobacterium-abscessus
#20
Katja Becker, Klara Haldimann, Petra Selchow, Lukas M Reinau, Michael Dal Molin, Peter Sander
Lipoproteins are important components of the mycobacterial cell envelope due to their function in cell wall homeostasis and bacterial virulence. They are post-translationally modified with lipid- and glycosyl-residues in various species and interference with acylation or glycosylation leads to reduced growth and attenuated virulence in Mycobacterium tuberculosis . Lipoproteins are also expressed in the emerging and highly drug resistant pathogen Mycobacterium abscessus which frequently affects the lungs of patients with chronic pulmonary disease or cystic fibrosis...
2017: Frontiers in Microbiology
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