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https://www.readbyqxmd.com/read/29274943/the-association-between-staphylococcus-aureus-and-subsequent-bronchiectasis-in-children-with-cystic-fibrosis
#1
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
#2
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...
December 15, 2017: 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
#3
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...
December 11, 2017: Journal of Medical Microbiology
https://www.readbyqxmd.com/read/29217601/novel-magnetic-resonance-technique-for-functional-imaging-of-cystic-fibrosis-lung-disease
#4
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
#5
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
#6
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
#7
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
#8
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
https://www.readbyqxmd.com/read/29151513/a-favorable-response-to-levetiracetam-in-a-patient-with-metastatic-adenoid-cystic-carcinoma
#9
Shinya Sakata, Sho Saeki, Yasuhiro Terasaki, Yoshihiro Natori, Kazuhiko Fujii
Adenoid cystic carcinoma (ACC) is a rare cancer, and there are no standard-of-care treatments for patients with metastatic ACC. We herein report a patient with lung metastasis of ACC who achieved a favorable response to levetiracetam. A 52-year-old Japanese man was admitted to our hospital because of multiple lung metastases of ACC. We performed first-line chemotherapy with cisplatin plus gemcitabine, and subsequently oral S-1 as second-line chemotherapy, which resulted in disease progression. The patient developed symptomatic epilepsy and received levetiracetam (250 mg twice daily)...
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29055982/vitamin-a-status-and-its-determinants-in-patients-with-cystic-fibrosis
#10
Ewa Sapiejka, Patrycja Krzyżanowska, Dariusz Walkowiak, Ewa Wenska-Chyży, Mariusz Szczepanik, Szczepan Cofta, Andrzej Pogorzelski, Wojciech Skorupa, Jarosław Walkowiak
BACKGROUND: Routine administration of vitamin A, recommended in CF patients, can help to prevent its deficiency. However, high vitamin A supplementation may lead to its excessive level and possible toxicity. Therefore, the aim of the present study was to assess the status of vitamin A and the determinants of its body resources in CF patients. METHODS: In 196 CF patients aged from 4 months to 47 years, the following parameters  were analysed: nutritional status (standardized body weight and height, serum albumin concentration) and clinical expression of disease (lung function - spirometry; biochemical markers of liver function - ALT, AST, GGT; respiratory tract colonization by Pseudomonas aeruginosa; diabetes; cirrhosis, non-cirrhotic liver disease; exocrine pancreatic function - fecal elastase-1 concentration; blood clotting -  INR  and  vitamin  A supplementation)...
July 2017: Acta Scientiarum Polonorum. Technologia Alimentaria
https://www.readbyqxmd.com/read/29046368/enhancement-of-lung-gene-delivery-after-aerosol-a-new-strategy-using-non-viral-complexes-with-antibacterial-properties
#11
REVIEW
Angélique Mottais, Tony Le Gall, Yann Sibiril, Julian Ravel, Véronique Laurent, Frédérique d'Arbonneau, Tristan Montier
The pathophysiology of obstructive pulmonary diseases, such as cystic fibrosis (CF), leads to the development of chronic infections in the respiratory tract. Thus, the symptomatic management of the disease requires, in particular, repetitive antibiotherapy. Besides these antibacterial treatments, certain pathologies, such as CF or chronic obstructive pulmonary disease (COPD), require the intake of many drugs. This simultaneous absorption may lead to undesirable drug interactions. For example, Orkambi® (lumacaftor/Ivacaftor, Vertex), a pharmacological drug employed to treat F508del patients, cannot be used with antibiotics such as rifampicin or rifabutin (rifamycin family) which are necessary to treat Mycobacteriaceae...
December 22, 2017: Bioscience Reports
https://www.readbyqxmd.com/read/28991007/the-optimal-approach-to-nutrition-and-cystic-fibrosis-latest-evidence-and-recommendations
#12
Francis M Hollander, Nicole M de Roos, Harry G M Heijerman
PURPOSE OF REVIEW: Cystic fibrosis (CF) is a progressive genetic disease that affects multiple organ systems. Therapy is directed to maintain and optimize nutritional status and pulmonary function, as these are key factors in survival. In this review, the most recent findings regarding nutritional management associated with pulmonary function and outcome will be explored. RECENT FINDINGS: Evidence-based and expert-based guidelines emphasize the need for adequate nutritional intake to improve nutritional status...
November 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28982773/inter-test-reproducibility-of-the-lung-clearance-index-measured-by-multiple-breath-washout
#13
Esther Oude Engberink, Felix Ratjen, Stephanie D Davis, George Retsch-Bogart, Reshma Amin, Sanja Stanojevic
The lung clearance index (LCI) has strong intra-test repeatability; however, the inter-test reproducibility of the LCI is poorly defined.The aim of the present study was to define a physiologically meaningful change in LCI in preschool children, which discriminates changes associated with disease progression from biological variability.Repeated LCI measurements from a longitudinal cohort study of children with cystic fibrosis and age-matched controls were collected to define the inter-visit reproducibility of the LCI...
October 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28960664/efficacy-and-safety-of-transbronchial-lung-biopsy-for-the-diagnosis-of-lymphangioleiomyomatosis-a-report-of-24-consecutive-patients
#14
Taro Koba, Toru Arai, Masanori Kitaichi, Takahiko Kasai, Masaki Hirose, Kazunobu Tachibana, Chikatoshi Sugimoto, Masanori Akira, Seiji Hayashi, Yoshikazu Inoue
BACKGROUND AND OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a diffuse cystic lung disease that occurs in women of childbearing age. LAM can be diagnosed on a clinical basis in patients with typical high-resolution computed tomography (HRCT) patterns and at least one other corroborating disease feature, such as chylothorax, angiomyolipoma, tuberous sclerosis complex or elevated serum vascular endothelial growth factor (VEGF)-D. However, patients who do not meet these criteria require tissue confirmation for a definitive diagnosis, and the utility of methods that are less invasive than surgical lung biopsy, such as transbronchial lung biopsy (TBLB), are not well studied...
September 28, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28950435/lung-clearance-index-lci-as-a-predictor-of-exercise-limitation-among-cf-patients
#15
Vasiliki Avramidou, Elpis Hatziagorou, Asterios Kampouras, Helge Hebestreit, Eleana Kourouki, Fotis Kirvassilis, John Tsanakas
INTRODUCTION: FEV1 is often considered the gold standard to monitor lung disease in cystic fibrosis (CF). Recently, there has been increasing interest in multiple breath washout (MBW) and cardiopulmonary exercise testing (CPET) as alternative or even more sensitive techniques. However, limited data exist on associations among the above methods. AIM: To evaluate the correlations between outcome measures of MBW and CPET and to examine if ventilation inhomogeneity can predict exercise intolerance...
September 26, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28934361/restriction-modification-mediated-barriers-to-exogenous-dna-uptake-and-incorporation-employed-by-prevotella-intermedia
#16
Christopher D Johnston, Chelsey A Skeete, Alexey Fomenkov, Richard J Roberts, Susan R Rittling
Prevotella intermedia, a major periodontal pathogen, is increasingly implicated in human respiratory tract and cystic fibrosis lung infections. Nevertheless, the specific mechanisms employed by this pathogen remain only partially characterized and poorly understood, largely due to its total lack of genetic accessibility. Here, using Single Molecule, Real-Time (SMRT) genome and methylome sequencing, bisulfite sequencing, in addition to cloning and restriction analysis, we define the specific genetic barriers to exogenous DNA present in two of the most widespread laboratory strains, P...
2017: PloS One
https://www.readbyqxmd.com/read/28899426/benign-metastasizing-leiomyoma-presenting-as-multiple-cystic-pulmonary-nodules-a-case-report
#17
Yeong Hun Choe, So Yeon Jeon, Yoon Chae Lee, Myung Ja Chung, Seung Yong Park, Yong Chul Lee, So Ri Kim
BACKGROUND: Benign metastatic leiomyoma (BML) is an extremely rare disease. Although uterine leiomyomas are benign histologically, they can metastasize to distant sites. While the incidence is very low, the lung is the organ most frequently affected by BML. Pulmonary BML usually presents as numerous well-defined nodules of various sizes, while the cavitary or cystic features in the nodules are rarely observed on radiologic images. CASE PRESENTATION: A 52-year-old woman complained of cough and dyspnea for one month...
September 12, 2017: BMC Women's Health
https://www.readbyqxmd.com/read/28871575/inert-gas-washout-background-and-application-in-various-lung-diseases
#18
Jakob Usemann, Sophie Yammine, Florian Singer, Philipp Latzin
Multiple breath inert gas washout (MBW) is a lung function technique to measure ventilation inhomogeneity. The technique was developed more than 60 years ago, but not much used for many decades. Technical improvements, easy protocols and higher sensitivity compared with standard lung function tests in some disease groups have led to a recent renaissance of MBW. The lung clearance index (LCI) is a common measure derived from MBW tests, and offers information on lung pathology complementary to that from conventional lung function tests such as spirometry...
September 5, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/28866642/the-quantitative-link-of-lung-clearance-index-to-bronchial-segments-affected-by-bronchiectasis
#19
Sylvia Verbanck, Gregory G King, Wenxiao Zhou, Anne Miller, Cindy Thamrin, Daniel Schuermans, Bart Ilsen, Caroline W Ernst, Johan de Mey, Walter Vincken, Eef Vanderhelst
In adult patients with cystic fibrosis (CF), the lung clearance index (LCI) derived from the multiple breath washout relates to both acinar and conductive ventilation heterogeneity. The latter component predicts an association between LCI and the number of bronchial segments affected by bronchiectasis. Here, we experimentally demonstrated this association in patients with CF, and also examined an ancillary group of patients with non-CF bronchiectasis. We conclude that lung disease severity in terms of number of bronchial segments results in an associated LCI increase, likely constituting a portion of LCI that cannot be reversed by treatment in patients with CF lung disease...
September 2, 2017: Thorax
https://www.readbyqxmd.com/read/28855057/cystic-fibrosis
#20
Susanne Naehrig, Cho-Ming Chao, Lutz Naehrlich
BACKGROUND: Universal screening of newborn babies for cystic fibrosis was launched in Germany on 1 September 2016. Here we present up-to-date information on the diagnosis, treatment, and prognosis of this disease. METHODS: This article is based on relevant publications retrieved by a selective search in PubMed, along with guidelines from Germany and abroad and systematic reviews. RESULTS: Cystic fibrosis is caused by a gene mutation leading to dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein...
August 21, 2017: Deutsches Ärzteblatt International
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