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https://www.readbyqxmd.com/read/28009030/long-term-outcome-of-cystic-fibrosis-patients-with-multisystem-evaluation
#1
Maryam Hassanzad, Mohammad Reza Boloursaz, Sepideh Darougar, Sabereh Tashayoie Nejad, Seyed Amir Mohajerani, Nooshin Baghaie, Seyed Karen Hashemitari, Ali Akbar Velayati
INTRODUCTION: Cystic fibrosis is a chronic disease with multiple organ involvement and chiefly results in chronic respiratory infections, pancreatic insufficiency and associated complications. The age at diagnosis, clinical presentation, rate of disease progression and prognosis is variable among patients. This study is designed to evaluate the behavior of disease to provide epidemiologic data for early recognition and proper management. MATERIAL AND METHODS: The study was designed as an active surveillance of 192 patients diagnosed with cystic fibrosis in a tertiary lung disease centre between 2008 and 2015...
2016: Adv Respir Med
https://www.readbyqxmd.com/read/27990603/sinonasal-characteristics-and-quality-of-life-by-snot-22-in-adult-patients-with-cystic-fibrosis
#2
Suzie Hyeona Kang, Camila Degen Meotti, Karine Bombardelli, Otávio Bejzman Piltcher, Paulo de Tarso Roth Dalcin
: The prevalence of chronic sinus disease in cystic fibrosis (CF) has gradually increased. Sinonasal involvement may have influence on pulmonary exacerbations and can have a negative impact on the quality of life. To evaluate nasal characteristics and quality of life in adult patients with CF; to establish an association and determine the predictors in SNOT-22 questionnaire. Cross- sectional study with prospective data collection was performed to evaluate adult CF patients. Patients underwent clinical evaluation, lung function tests, nasal endoscopy, and paranasal sinuses CT scan...
December 18, 2016: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/27943680/progression-of-lung-disease-in-preschool-patients-with-cystic-fibrosis
#3
Sanja Stanojevic, Stephanie D Davis, George Retsch-Bogart, Hailey Webster, Miriam Davis, Robin C Johnson, Renee Jensen, Maria Ester Pizarro, Mica Kane, Charles C Clem, Leah Schornick, Padmaja Subbarao, Felix A Ratjen
RATIONALE: Implementation of intervention strategies to prevent lung damage in early cystic fibrosis (CF) requires objective outcome measures that capture and track lung disease. OBJECTIVE: To define the utility of the Lung Clearance Index (LCI), measured by Multiple Breath Washout (MBW), as a means to track disease progression in preschool children with CF. METHODS: CF children between the ages of 2.5 and 6 years with a confirmed diagnosis of CF and age-matched healthy controls were enrolled at three North American CF centers...
December 12, 2016: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/27927050/the-use-of-multiple-breath-washout-for-assessing-cystic-fibrosis-in-infants
#4
Gwyneth Davies, Paul Aurora
Lung Clearance Index, measured using the multiple breath washout (MBW) technique, may be a useful test in infants with Cystic Fibrosis (CF). However, the requirement for specialised equipment and a number of important technical and methodological considerations relevant to testing in infants have complicated matters, and to date prevented its widespread translatability in this age group. Areas covered: We review the current status of infant MBW testing in CF, focusing on Lung Clearance Index. This includes a review of recent developments in the field relevant to testing methodology in the infant population, use in evaluating lung disease in CF in infancy, and the associated challenges which remain...
January 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/27902340/viral-bacterial-interactions-in-the-respiratory-tract
#5
Carla Bellinghausen, Gernot G U Rohde, Paul H M Savelkoul, Emiel F M Wouters, Frank R M Stassen
In the respiratory tract, viruses and bacteria can interact on multiple levels. It is well known that respiratory viruses, particularly influenza viruses, increase the susceptibility to secondary bacterial infections. Numerous mechanisms, including compromised physical and immunological barriers, and changes in the microenvironment have hereby been shown to contribute to the development of secondary bacterial infections. In contrast, our understanding of how bacteria shape a response to subsequent viral infection is still limited...
December 2016: Journal of General Virology
https://www.readbyqxmd.com/read/27870729/aggressive-digital-papillary-adenocarcinoma-with-multiple-organ-metastases-a-case-report-and-review-of-the-literature
#6
Tadahiro Kobayashi, Azusa Hiura, Kyosuke Oishi, Shintaro Maeda, Andres James Le Pavoux, Kuniaki Ohara, Hironori Uruga
Aggressive digital papillary adenocarcinoma (ADPA) is a rare sweat gland neoplasm with a high recurrence rate and metastatic potential. In this study, the authors describe a case that originally appeared to benign spiradenoma, but took an ominous course eventually resulting in the diagnosis of ADPA. A 73-year-old woman developed a gradually growing nodule on the second toe of her left foot, which she had first noticed 4 years previously. An excisional biopsy was performed followed by histological examination...
December 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27863009/regulation-of-mrna-turnover-in-cystic-fibrosis-lung-disease
#7
REVIEW
Roopa Biswas, Parameet Kumar, Harvey B Pollard
Cystic fibrosis (CF) is an autosomal recessive disease due to mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, F508del-CFTR being the most frequent mutation. The CF lung is characterized by a hyperinflammatory phenotype and is regulated by multiple factors that coordinate its pathophysiology. In CF the expression of CFTR as well as proinflammatory genes are regulated at the level of messenger RNA (mRNA) stability, which subsequently affect translation. These mechanisms are mediated by inflammatory RNA-binding proteins as well as small endogenous noncoding microRNAs, in coordination with cellular signaling pathways...
November 13, 2016: Wiley Interdisciplinary Reviews. RNA
https://www.readbyqxmd.com/read/27822534/long-term-evolution-of-burkholderia-multivorans-during-a-chronic-cystic-fibrosis-infection-reveals-shifting-forces-of-selection
#8
Inês N Silva, Pedro M Santos, Mário R Santos, James E A Zlosnik, David P Speert, Sean W Buskirk, Eric L Bruger, Christopher M Waters, Vaughn S Cooper, Leonilde M Moreira
Burkholderia multivorans is an opportunistic pathogen capable of causing severe disease in patients with cystic fibrosis (CF). Patients may be chronically infected for years, during which the bacterial population evolves in response to unknown forces. Here we analyze the genomic and functional evolution of a B. multivorans infection that was sequentially sampled from a CF patient over 20 years. The population diversified into at least four primary, coexisting clades with distinct evolutionary dynamics. The average substitution rate was only 2...
May 2016: MSystems
https://www.readbyqxmd.com/read/27811936/transglutaminase-2-is-dispensable-but-required-for-the-survival-of-mice-in-dextran-sulfate-sodium-induced-colitis
#9
Eui Man Jeong, Young Hoon Son, Yewon Choi, Jin-Hee Kim, Jin-Haeng Lee, Sung-Yup Cho, In-Gyu Kim
Transglutaminase 2 (TG2) is a ubiquitously expressed enzyme that catalyzes crosslinking, polyamination or deamidation of glutamine residues in proteins. It has been reported that TG2 is involved in the pathogenesis of various inflammatory diseases including celiac disease, pulmonary fibrosis, cystic fibrosis, multiple sclerosis and sepsis. Recently, using a mouse model of bleomycin-induced lung fibrosis, we showed that TG2 is required to trigger inflammation via the induction of T helper type 17 (Th17) cell differentiation in response to tissue damage...
November 4, 2016: Experimental & Molecular Medicine
https://www.readbyqxmd.com/read/27754353/the-impact-of-secondhand-smoke-exposure-on-children-with-cystic-fibrosis-a-review
#10
Benjamin T Kopp, Juan Antonio Ortega-García, S Christy Sadreameli, Jack Wellmerling, Estelle Cormet-Boyaka, Rohan Thompson, Sharon McGrath-Morrow, Judith A Groner
Secondhand smoke exposure (SHSe) has multiple adverse effects on lung function and growth, nutrition, and immune function in children; it is increasingly being recognized as an important modifier of disease severity for children with chronic diseases such as cystic fibrosis (CF). This review examines what is known regarding the prevalence of SHSe in CF, with the majority of reviewed studies utilizing parental-reporting of SHSe without an objective biomarker of exposure. A wide range of SHSe is reported in children with CF, but under-reporting is common in studies involving both reported and measured SHSe...
October 12, 2016: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/27730195/the-cystic-fibrosis-lower-airways-microbial-metagenome
#11
Patricia Moran Losada, Philippe Chouvarine, Marie Dorda, Silke Hedtfeld, Samira Mielke, Angela Schulz, Lutz Wiehlmann, Burkhard Tümmler
Chronic airway infections determine most morbidity in people with cystic fibrosis (CF). Herein, we present unbiased quantitative data about the frequency and abundance of DNA viruses, archaea, bacteria, moulds and fungi in CF lower airways. Induced sputa were collected on several occasions from children, adolescents and adults with CF. Deep sputum metagenome sequencing identified, on average, approximately 10 DNA viruses or fungi and several hundred bacterial taxa. The metagenome of a CF patient was typically found to be made up of an individual signature of multiple, lowly abundant species superimposed by few disease-associated pathogens, such as Pseudomonas aeruginosa and Staphylococcus aureus, as major components...
April 2016: ERJ Open Research
https://www.readbyqxmd.com/read/27718754/special-considerations-for-the-treatment-of-pulmonary-exacerbations-in-children-with-cystic-fibrosis
#12
Valerie Waters, Sanja Stanojevic, Felix Ratjen
Introduction Cystic fibrosis (CF) is a disease characterized by recurrent flares of respiratory symptoms, known as pulmonary exacerbations (PExs), which have a cumulative, detrimental effect on lung function decline and overall mortality. Although much research has been done on the effects of PExs in adults with CF, considerably less is known about these events in young children with CF. Areas covered This review describes the typical presentation of PExs in children and their impact on long-term clinical outcomes...
October 8, 2016: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/27686146/population-pharmacokinetics-of-mycophenolic-acid-and-its-glucuronide-metabolite-in-lung-transplant-recipients-with-and-without-cystic-fibrosis
#13
Xiao-Xing Wang, Wenfang Liu, Tian Zheng, Jeong M Park, David E Smith, Meihua R Feng
1. Cystic fibrosis (CF) is a disease affecting multiple organs that may reduce the systemic exposure of some drugs. The objective of this work was to characterize and compare the population pharmacokinetics (PK) of the immunosuppressant mycophenolic acid (MPA), and its glucuronide metabolite (MPAG) in adult lung transplant recipients with and without CF (NCF) following repeated oral administration of the prodrug mycophenolate mofetil (MMF). 2. A population PK model was developed, with simultaneous modeling of MPA and MPAG, using nonlinear mixed effects modeling...
August 10, 2016: Xenobiotica; the Fate of Foreign Compounds in Biological Systems
https://www.readbyqxmd.com/read/27684048/burkholderia-cenocepacia-k56-2-trimeric-autotransporter-adhesin-bcaa-binds-tnfr1-and-contributes-to-induce-airway-inflammation
#14
Dalila Mil-Homens, Sandra N Pinto, Rute G Matos, Cecília Arraiano, Arsenio M Fialho
Chronic lung disease caused by persistent bacterial infections is a major cause of morbidity and mortality in patients with cystic fibrosis (CF). CF pathogens acquire antibiotic resistance, overcame host defenses and impose uncontrolled inflammation that ultimately may cause permanent damage of lungs' airways. Among the multiple CF-associated pathogens, Burkholderia cenocepacia and other B. cepacia complex (Bcc) bacteria have become prominent contributors of disease progression. Here, we demonstrate that BcaA, a trimeric autotransporter adhesin (TAA) from the epidemic strain B...
September 29, 2016: Cellular Microbiology
https://www.readbyqxmd.com/read/27645685/lung-clearance-index-lci-in-patients-with-bronchiolitis-obliterans-a-preliminary-report-and-comparison-to-cystic-fibrosis-patients
#15
Michal Gur, Karin Yaacoby-Bianu, Anat Ilivitzki, Ronen Bar-Yoseph, Vered Nir, Fahed Hakim, Yazeed Toukan, Lea Bentur
INTRODUCTION: Bronchiolitis obliterans (BO) is a chronic airway disease following an insult to the lower respiratory tract. Lung clearance index (LCI) measures ventilation inhomogeneity and has been studied in cystic fibrosis (CF). We aimed to evaluate LCI in BO and to compare it to LCI in CF patients. METHODS: LCI was measured in BO patients, compared to CF patients, and correlated with spirometry and CT findings. RESULTS: Twenty BO patients and 26 CF patients (with similar mean age and BMI) underwent evaluation...
August 31, 2016: Lung
https://www.readbyqxmd.com/read/27609088/-extrahepatic-and-extrapulmonary-hydatidosis
#16
Farah Adel, José Manuel Ramia, Luis Gijón, Roberto de la Plaza-Llamas, Vladimir Arteaga-Peralta, Carmen Ramiro-Perez
BACKGROUND: Cystic echinococcosis is a zoonosis caused by larvae of the parasite Echinococcus that is endemic in many countries of the Mediterranean area. It can affect any organ, with the most common sites being liver (70%) and lung (20%). Splenic hydatid disease, despite being rare, is the third most common location. Other locations such as bone, skin, or kidney are exceptional. OBJECTIVE: To present our experience in extrahepatic and extrapulmonary hydatidosis...
September 5, 2016: Cirugia y Cirujanos
https://www.readbyqxmd.com/read/27605826/histopathological-changes-associated-with-e-granulosus-echinococcosis-in-food-producing-animals-in-punjab-india
#17
B B Singh, R Sharma, J K Sharma, V Mahajan, J P S Gill
Cystic echinococcosis due to Echinococcus granulosus is a serious public health and economic concern in India. The disease is endemic in most of the food producing animals such as cattle, buffalo, sheep, goat and pigs in the country. In this study, tissues comprising of pieces of liver and lungs were collected in 10 % formal buffered saline. The formalin fixed tissues (liver and lungs) from 10 cattle, buffalo, sheep, goat and pigs each were selected and further processed by acetone benzene method for histopathological examinations...
September 2016: Journal of Parasitic Diseases: Official Organ of the Indian Society for Parasitology
https://www.readbyqxmd.com/read/27592691/minimal-clinically-important-difference-for-change-in-6-minute-walk-test-distance-of-adults-with-pathology-a-systematic-review
#18
Richard W Bohannon, Rebecca Crouch
RATIONALE, AIMS, AND OBJECTIVES: The 6-minute walk test (6MWT) is widely used as a test of functional exercise capacity. Several studies have reported the minimal clinically important difference (MCID) for the 6MWT; however, the findings of the studies have not been examined in the context of one another. In this review, we aimed to summarize available information on the MCID for the 6MWT performed by patients with pathology. METHODS: Relevant literature was identified by searches of 3 electronic databases (PubMed, Scopus, and Cumulative Index of Nursing and Allied Health), examination of article reference lists, and consultation with an expert...
September 4, 2016: Journal of Evaluation in Clinical Practice
https://www.readbyqxmd.com/read/27576234/respiratory-conditions-update-cystic-fibrosis
#19
REVIEW
Lyle L Pritchard
Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction...
September 2016: FP Essentials
https://www.readbyqxmd.com/read/27575911/comparison-of-lung-clearance-index-and-magnetic-resonance-imaging-for-assessment-of-lung-disease-in-children-with-cystic-fibrosis
#20
Mirjam Stahl, Mark O Wielpütz, Simon Y Graeber, Cornelia Joachim, Olaf Sommerburg, Hans-Ulrich Kauczor, Michael Puderbach, Monika Eichinger, Marcus A Mall
RATIONALE: Early onset and progression of lung disease in children with cystic fibrosis (CF) indicates that sensitive noninvasive outcome measures are needed for diagnostic monitoring and early intervention clinical trials. The lung clearance index (LCI) and chest magnetic resonance imaging (MRI) were shown to detect early lung disease in CF, however, the relationship between the two measures remains unknown. OBJECTIVES: To correlate the LCI with abnormalities detected by MRI and compare the sensitivity of the two techniques to detect responses to therapy for pulmonary exacerbations in children with CF...
August 30, 2016: American Journal of Respiratory and Critical Care Medicine
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