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https://www.readbyqxmd.com/read/28220034/exhaled-and-nasal-nitric-oxide-in-relation-to-lung-function-blood-cell-counts-and-disease-characteristics-in-cystic-fibrosis
#1
Christina Krantz, Christer Janson, Annika Hollsing, Kjell Alving, Andrei Malinovschi
BACKGROUND: Patients with CF have similar or lower exhaled nitric oxide (FeNO) and lower nasal nitric oxide (nNO) levels than controls. There are divergent results on alveolar NO (CalvNO) concentrations in relation to CF. There are inconsistent results on correlation between different nitric oxide parameters and lung function and inflammation in CF. AIM: To compare FeNO, CalvNO and nNO levels between subjects with CF, asthma and healthy controls and to study whether these parameters are related to lung function, blood cell counts or clinical characteristics in CF patients...
February 21, 2017: Journal of Breath Research
https://www.readbyqxmd.com/read/28203428/multiple-cavitating-pulmonary-nodules-rare-manifestation-of-benign-metastatic-leiomyoma
#2
Sa Ra Lee, Youn-I Choi, Seok Jeong Lee, Sung Shine Shim, Jin Hwa Lee, Yoo Kyung Kim, Soon Hee Sung
Benign metastasizing leiomyoma (BML) is a rare disease of pathologically benign, but the tumor metastasizes to other organs. The most common organ involved in BML is lung. Pulmonary involvement usually manifested as multiple nodules on chest X-ray, however we experienced an interesting case of a 52-year-old premenopausal woman who presented with multiple bilateral lung cavitations and nodules on a chest X-ray without any respiratory symptoms. Chest computed-tomography identified multiple cavitary lesions of 5-12 mm in diameter and well-defined nodules of 5-10 mm in diameter in both lung fields...
January 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28188011/effect-of-posture-on-lung-ventilation-distribution-and-associations-with-structure-in-children-with-cystic-fibrosis
#3
Kathryn A Ramsey, Caroline McGirr, Stephen M Stick, Graham L Hall, Shannon J Simpson
BACKGROUND: We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease. METHODS: Multiple breath washout (MBW) was performed in seated and supine postures in 25 healthy children and 21 children with CF. Children with CF also underwent a chest CT scan. Functional residual capacity (FRC), lung clearance index (LCI) and moment ratios were calculated from the MBW test. CT scans were evaluated for CF-related structural lung disease...
February 7, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28174186/avoiding-misdiagnosis-cystic-calcified-brain-metastases-of-uterine-cervical-cancer-mimicking-neurocysticercosis
#4
Jacopo Fantini, Arianna Sartori, Paolo Manganotti
The radiological finding of multiple calcified brain lesions is atypical for brain metastases and in absence of a clear evidence of disseminated neoplastic disease the differential diagnosis may be difficult. Calcified brain metastases (CBM) are rarely encountered in clinical practice and they mostly arise from lung, breast and gastrointestinal primitive tumours. Only one case of uterine cervical carcinoma (UCC) with CBM has been reported so far. We describe the case of a 41-year-old Caucasian woman with a history of hysterectomy and bilateral salpingo-oophorectomy for UCC 3 years prior to observation and no evidence of neoplastic recurrence that developed cystic CBM...
February 7, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28158236/pro-resolving-lipid-mediator-resolvin-d1-serves-as-a-marker-of-lung-disease-in-cystic-fibrosis
#5
Olaf Eickmeier, Daniela Fussbroich, Klaus Mueller, Friederike Serve, Christina Smaczny, Stefan Zielen, Ralf Schubert
BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive genetic disorder that affects multiple organs, including the lungs, pancreas, liver and intestine. Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) locus lead to defective proteins and reduced Cl- secretion and Na+ hyperabsorption in the affected organs. In addition, patients suffering from CF display chronic inflammation that contributes to the pathogenesis of CF. Recent work suggests that CF patients have a reduced capacity to biosynthesize specialized pro-resolving lipid mediators (SPMs), which contributes to the development and duration of the unwanted inflammation...
2017: PloS One
https://www.readbyqxmd.com/read/28116329/cftr-founder-mutation-causes-protein-trafficking-defects-in-chinese-patients-with-cystic-fibrosis
#6
Gordon K C Leung, Dingge Ying, Christopher C Y Mak, Xin-Ying Chen, Weiyi Xu, Kit-San Yeung, Wai-Lap Wong, Yoyo W Y Chu, Gary T K Mok, Christy S K Chau, Jenna McLuskey, Winnie P T Ong, Huey-Yin Leong, Kelvin Y K Chan, Wanling Yang, Jeng-Haur Chen, Albert M Li, Pak C Sham, Yu-Lung Lau, Brian H Y Chung, So-Lun Lee
BACKGROUND: Cystic fibrosis (CF) is a rare condition in Asians. Since 1985, only about 30 Chinese patients have been reported with molecular confirmation. METHOD: Using our in-house next-generation sequencing (NGS) pipeline for childhood bronchiectasis, we identified disease-causing CFTR mutations in CF patients in Hong Kong. After identifying p.I1023R in multiple patients, haplotype analysis was performed with genome-wide microarray to ascertain the likelihood of this being a founder mutation...
January 2017: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/28096282/the-effect-of-mtor-inhibitors-on-respiratory-infections-in-lymphangioleiomyomatosis
#7
REVIEW
Andrew M Courtwright, Hilary J Goldberg, Elizabeth Petri Henske, Souheil El-Chemaly
Lymphangioleiomyomatosis (LAM) is a destructive cystic lung disease. Mammalian target of rapamycin (mTOR) inhibitors are the primary treatment for LAM but it is unknown whether these immunosuppressing medications increase the risk for or the severity of respiratory infections in LAM patients.We searched multiple databases for original articles that reported the rate of respiratory infections in LAM patients treated with mTOR inhibitors or placebo. We calculated incidence rates for respiratory infections in these groups and incidence rate ratios for respiratory infections and severe respiratory infections in mTOR inhibitors treated versus placebo treated patients...
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28009030/long-term-outcome-of-cystic-fibrosis-patients-with-multisystem-evaluation
#8
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
#9
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
#10
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
#11
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
#12
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
#13
REVIEW
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
#14
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
#15
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
#16
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
#17
REVIEW
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
#18
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
#19
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
#20
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
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