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Idiopathic pulmonary disease

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https://www.readbyqxmd.com/read/29150411/an-epithelial-biomarker-signature-for-idiopathic-pulmonary-fibrosis-an-analysis-from-the-multicentre-profile-cohort-study
#1
Toby M Maher, Eunice Oballa, Juliet K Simpson, Joanne Porte, Anthony Habgood, William A Fahy, Aiden Flynn, Philip L Molyneaux, Rebecca Braybrooke, Hrushikesh Divyateja, Helen Parfrey, Doris Rassl, Anne-Marie Russell, Gauri Saini, Elisabetta A Renzoni, Anne-Marie Duggan, Richard Hubbard, Athol U Wells, Pauline T Lukey, Richard P Marshall, R Gisli Jenkins
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal disorder with a variable disease trajectory. The aim of this study was to assess potential biomarkers to predict outcomes for people with IPF. METHOD: PROFILE is a large prospective longitudinal cohort of treatment-naive patients with IPF. We adopted a two-stage discovery and validation design using patients from the PROFILE cohort. For the discovery analysis, we examined 106 patients and 50 age and sex matched healthy controls from Nottingham University Hospitals NHS Trust and the Royal Brompton Hospital...
November 14, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/29150159/diagnostic-performance-of-pulmonary-capacitance-at-rest-and-during-exercise-in-idiopathic-pulmonary-arterial-hypertension
#2
Pankaj Jain, Sriram Rao, Peter Macdonald, Eugene Kotlyar, Andrew Jabbour, Christopher Hayward, Anne Keogh
BACKGROUND: Reliable markers of early disease are needed in pulmonary arterial hypertension (PAH). As measures of the contribution of abnormal vascular compliance to overall vascular resistance, resting and exercise pulmonary capacitance-defined as the stroke volume divided by the change in pulmonary pulse pressure-may be sensitive markers of early disease. METHODS: We examined all patients in our pulmonary hypertension database with idiopathic PAH, who had undergone rest and exercise right heart catheterisation in one sitting...
November 8, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/29148977/the-role-of-micrornas-in-chronic-respiratory-disease-recent-insights
#3
Lindsay R Stolzenburg, Ann Harris
Chronic respiratory diseases encompass a group of diverse conditions affecting the airways, which all impair lung function over time. They include cystic fibrosis, idiopathic pulmonary fibrosis, chronic obstructive pulmonary disease, and asthma, which together affect hundreds of millions of people worldwide. MicroRNAs (miRNAs), a class of small non-coding RNAs involved in post-transcriptional gene repression, are now recognized as major regulators in the development and progression of chronic lung disease. Alterations in miRNA abundance occur in lung tissue, inflammatory cells, and freely circulating in blood and are thought to function both as drivers and modifiers of disease...
November 27, 2017: Biological Chemistry
https://www.readbyqxmd.com/read/29147917/recommendations-for-perioperative-management-of-lung-cancer-patients-with-comorbidities
#4
REVIEW
Hiroyoshi Tsubochi, Tomoki Shibano, Shunsuke Endo
OBJECTIVES: To improve surgical outcomes, clinicians must provide optimal perioperative care for comorbidities identified as significant factors in risk models for patients undergoing lung cancer surgery. METHODS: We reviewed trends in perioperative care for idiopathic pulmonary fibrosis, cardiovascular diseases, and end-stage renal diseases in patients undergoing lung cancer surgery, as large clinical databases indicate that these comorbidities are significant risk factors for lung cancer surgery...
November 16, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29146100/emerging-role-of-extracellular-vesicles-as-a-senescence-associated-secretory-phenotype-insights-into-the-pathophysiology-of-lung-diseases
#5
REVIEW
Tsukasa Kadota, Yu Fujita, Yusuke Yoshioka, Jun Araya, Kazuyoshi Kuwano, Takahiro Ochiya
Aging is a major risk factor for the development of chronic lung diseases such as chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF), and lung cancer. A main aspect of aging is the impaired function of maintaining homeostasis in the organs and body, which is associated with cellular senescence. Cellular senescence is recognized as the state of irreversible cell cycle arrest in response to a variety of cellular stresses. Senescent cells are not simply cell cycle-arrested cells; they also affect bystander cells through the secretion of bioactive molecules, termed the senescence-associated secretory phenotype (SASP)...
November 13, 2017: Molecular Aspects of Medicine
https://www.readbyqxmd.com/read/29145311/pulmonary-hypertension-as-a-manifestation-of-mitochondrial-disease-a-case-report-and-review-of-the-literature
#6
Shan Xu, Xiaoling Xu, Jisong Zhang, Kejing Ying, Yuquan Shao, Ruifeng Zhang
BACKGROUND: Mitochondrial diseases are a group of multisystem heterogeneous diseases caused by pathologic dysfunction of the mitochondrial respiratory chain. A wide range of clinical expression has been described. However, pulmonary hypertension has rarely been described in association with mitochondrial disease until the past decade, and there is no currently recognized treatment for the pulmonary hypertension complicated with mitochondrial disorder. PATIENT CONCERNS: We reported the case of a 15-year-old boy who presented with shortness of breath and exercise limitation after a cold, and the diagnosis of pulmonary hypertension was confirmed by right heart catheter...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29144435/dysregulated-collagen-homeostasis-by-matrix-stiffening-and-tgf-%C3%AE-1-in-fibroblasts-from-idiopathic-pulmonary-fibrosis-patients-role-of-fak-akt
#7
Alícia Giménez, Paula Duch, Marta Puig, Marta Gabasa, Antoni Xaubet, Jordi Alcaraz
Idiopathic pulmonary fibrosis (IPF) is an aggressive disease in which normal lung parenchyma is replaced by a stiff dysfunctional scar rich in activated fibroblasts and collagen-I. We examined how the mechanochemical pro-fibrotic microenvironment provided by matrix stiffening and TGF-β1 cooperates in the transcriptional control of collagen homeostasis in normal and fibrotic conditions. For this purpose we cultured fibroblasts from IPF patients or control donors on hydrogels with tunable elasticity, including 3D collagen-I gels and 2D polyacrylamide (PAA) gels...
November 16, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29140119/ct-features-of-the-usual-interstitial-pneumonia-pattern-differentiating-connective-tissue-disease-associated-interstitial-lung-disease-from-idiopathic-pulmonary-fibrosis
#8
Jonathan H Chung, Christian W Cox, Steven M Montner, Ayodeji Adegunsoye, Justin M Oldham, Aliya N Husain, Rekha Vij, Imre Noth, David A Lynch, Mary E Strek
OBJECTIVE: A substantial proportion of cases of usual interstitial pneumonia (UIP) are due to connective tissue disease (CTD)-associated interstitial lung disease (ILD). The purpose of this study was to determine whether specific CT findings can help differentiate a UIP pattern of CTD-ILD from a UIP pattern of idiopathic pulmonary fibrosis (IPF) and whether these signs are associated with survival. MATERIALS AND METHODS: Adults visiting an ILD clinic from 2006 to 2015 enrolled in a research registry with a multidisciplinary diagnosis of CTD-ILD or IPF and a UIP pattern at high-resolution CT were included in the study...
November 15, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/29139354/-idiopathic-pulmonary-fibrosis-is-an-overlooked-disease
#9
EDITORIAL
Elisabeth Bendstrup, Sissel Kronborg-White, Thomas Skovhus Prior, Janne Møller, Charlotte Hyldgaard
Idiopathic pulmonary fibrosis is a chronic, progressive and fatal disease which primarily occurs in male patients over 60 years with a smoking history. Cryobiopsy is a new promising method for obtaining lung tissue for histologic analysis with fewer complications than surgical lung biopsy. Cryobiopsy allows more patients to be diagnosed. Antifibrotic treatment has shown to decrease progression and prolong survival time in both early and later stages of the disease. Early diagnosis and treatment are therefore of great importance to prevent disease progression and reduce mortality...
November 13, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/29139195/phenotypic-characteristics-associated-with-slow-gait-speed-in-idiopathic-pulmonary-fibrosis
#10
Claire M Nolan, Matthew Maddocks, Toby M Maher, Jane L Canavan, Sarah E Jones, Ruth E Barker, Suhani Patel, Joseph Jacob, Paul Cullinan, William D-C Man
BACKGROUND AND OBJECTIVE: Usual gait speed over 4 m (4MGS) is an established functional performance measure in older adults that consistently predicts adverse health outcomes, but few data exist in idiopathic pulmonary fibrosis (IPF). We assessed the reliability of 4MGS, its relationship with established outcome measures and its responsiveness to pulmonary rehabilitation. METHODS: In four prospective IPF cohorts, 4MGS inter-observer (n = 46) and test-retest (n = 46) reliability, concurrent validity (n = 65 and n = 62) and responsiveness (n = 60) were determined...
November 14, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29135404/-bilateral-total-lung-lavage-in-a-patient-with-pulmonary-alveolar-proteinosis
#11
Müge Erbay, Yılmaz Bülbül, Funda Öztuna, Mehmet Kılıç, Neslihan Özçelik, Şafak Ersöz
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant composed of proteins and lipids. Three main categories of PAP have been defined depending on the aetiology: primer/idiopathic, neonatal/congenital, secondary and exogenous/environmental exposure. Radiologically diffuse ground glass opacities, interlobular and intralobular septal thickening is seen. Although open lung biopsy is accepted as the gold standard in diagnosis, it can be diagnosed by showing bronchoalveolar lavage (BAL) fluid with a milky appearance and periodic acid-schiff (PAS) positive globules in biopsy with clinical and radiological findings...
September 2017: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/29135402/-schistosomiasis-and-pulmonary-hypertension
#12
Nazan Şen
Schistosomiasis is one of the most prevelant parazitic diseases in the world. It is endemic in more than 70 countries, and more than 200 million people worldwide are infected with Schistosoma. Pulmonary hypertension (PHT) is one of the chronic complications of schistosomiasis. The exact pathogenesis of schistosomiasis-associated pulmonary hypertension (S-PHT) remains unclear, although several mechanisms such as parazitic arterial embolisation, pulmonary arteriopathy, and portopulmonary hypertension have been suggested...
September 2017: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/29135399/-investigation-of-parameters-related-to-prognosis-in-diffuse-parenchymal-lung-diseases-prognosis-in-interstitial-lung-diseases
#13
Coşkun Canıvar, Züleyha Bingöl, Zeki Kılıçaslan, Tülin Çağatay, N Gülfer Okumuş
Introduction: Parameters related to prognosis in diffuse parenchymal lung disease (DPLD) have a decisive influence on treatment and follow-up processes. We aimed to define baseline characteristics and factors that effect the mortality of the group of patients with DPLD and to determine distinctions between subgroups. Materials and Methods: Demographic characteristics, complaints, comorbidity, treatment, pulmonary function tests, echocardiographic findings, six minute walking test (6MWT), arterial blood gases analysis, radiological findings and survival time were collected from outpatient clinics database...
September 2017: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/29129842/association-of-perioperative-redox-balance-on-long-term-outcome-in-patients-undergoing-lung-resection
#14
Osamu Araki, Yuji Matsumura, Takashi Inoue, Yoko Karube, Sumiko Maeda, Satoru Kobayashi, Masayuki Chida
PURPOSE: We examined whether redox balance during the perioperative period is associated with long-term survival of patients after undergoing lung resection. METHODS: Patients who underwent surgery for lung cancer from January to June 2013 at our institution were investigated. Serum was collected during the operation, and on postoperative day (POD) 3 and 7, and the levels of derivatives of reactive oxygen metabolites (d-ROMs) and biologic antioxidant potential (BAP) were measured...
November 10, 2017: Annals of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29126829/palliative-care-in-diffuse-interstitial-lund-disease-results-of-a-spanish-survey
#15
Silvia Barril, Ana Alonso, José Antonio Rodríguez-Portal, Margarita Viladot, Jordi Giner, Francisco Aparicio, Ana Romero-Ortiz, Orlando Acosta, Diego Castillo
INTRODUCTION: Interstitial lung diseases (ILD) and, in particular, idiopathic pulmonary fibrosis, may have a significant impact on patient survival. Recent studies highlight the need for palliative care (PC) in the management of ILD patients. The aim of this study was to determine the current situation of PC in patients in Spain. METHODS: A 36-question survey addressing the main aspects of PC in ILD patients was designed. The survey was sent via email to all members of the Spanish Society of Pulmonology and Thoracic Surgery...
November 7, 2017: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/29125826/lysyl-oxidases-regulate-fibrillar-collagen-remodelling-in-idiopathic-pulmonary-fibrosis
#16
Gavin Tjin, Eric S White, Alen Faiz, Delphine Sicard, Daniel J Tschumperlin, Annabelle Mahar, Eleanor P W Kable, Janette K Burgess
Idiopathic pulmonary fibrosis (IPF) is a progressive scarring disease of the lung with few effective therapeutic options. Structural remodelling of the extracellular matrix [i.e. collagen cross-linking mediated by the lysyl oxidase (LO) family of enzymes (LOX, LOXL1-4)] might contribute to disease pathogenesis and represent a therapeutic target. This study aimed to further our understanding of the mechanisms by which LO inhibitors might improve lung fibrosis. Lung tissues from IPF and non-IPF subjects were examined for collagen structure (second harmonic generation imaging) and LO gene (microarray analysis) and protein (immunohistochemistry and western blotting) levels...
November 1, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29125071/clinical-experience-in-idiopathic-pulmonary-fibrosis-a-retrospective-study
#17
Julien Guiot, Bernard Duysinx, Laurence Seidel, Monique Henket, Fanny Gester, Olivier Bonhomme, Jean-Louis Corhay, Renaud Louis
Introduction Idiopathic pulmonary fibrosis (IPF) is a rare lung disease with an increased incidence since the last few years. Here, we report our eight-year clinical experience in CHU of Liège, Belgium. Methods We have studied retrospectively patients recruited from our ambulatory care polyclinic at CHU of Liège from 1 January 2009 to 1 January 2017. We have excluded all patients treated with a specific anti-fibrotic therapy due to incomplete follow-up. The diagnosis of IPF was made according to the ATS/ERS international recommendations (2015)...
November 10, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/29123445/acute-exacerbation-of-idiopathic-pulmonary-fibrosis
#18
REVIEW
Tomoo Kishaba
Idiopathic pulmonary fibrosis (IPF) is the most common cause of chronic diffuse parenchymal disease of unknown cause. However, IPF patients sometimes develop acute exacerbation (AE), which is a life-threatening condition. The cause of AE of IPF remains unknown. The new criteria for AE of IPF have been proposed last year, wherein both idiopathic and triggered AE were proposed. Triggered AE includes infection, post-procedure and post-operation, drug toxicity, and aspiration. Therefore, detailed history taking is crucial...
October 2017: Eurasian Journal of Medicine
https://www.readbyqxmd.com/read/29122916/task-1-kcnk3-channels-in-the-lung-from-cell-biology-to-clinical-implications
#19
REVIEW
Andrea Olschewski, Emma L Veale, Bence M Nagy, Chandran Nagaraj, Grazyna Kwapiszewska, Fabrice Antigny, Mélanie Lambert, Marc Humbert, Gábor Czirják, Péter Enyedi, Alistair Mathie
TWIK-related acid-sensitive potassium channel 1 (TASK-1 encoded by KCNK3) belongs to the family of two-pore domain potassium channels. This gene subfamily is constitutively active at physiological resting membrane potentials in excitable cells, including smooth muscle cells, and has been particularly linked to the human pulmonary circulation. TASK-1 channels are sensitive to a wide array of physiological and pharmacological mediators that affect their activity such as unsaturated fatty acids, extracellular pH, hypoxia, anaesthetics and intracellular signalling pathways...
November 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29122755/renin-angiotensin-system-regulates-pulmonary-arterial-smooth-muscle-cell-migration-in-chronic-thromboembolic-pulmonary-hypertension
#20
Yun Xia Zhang, Ji Feng Li, Yuan Hua Yang, Zhen Guo Zhai, Song Gu, Yan Liu, Ran Miao, Ping Ping Zhong, Ying Wang, Xiao Xi Huang, Chen Wang
Pulmonary arterial smooth muscle cell (PASMC) migration plays a key role in vascular remodeling which occurs during development of chronic thromboembolic pulmonary hypertension (CTEPH). Activation of the renin-angiotensin system (RAS) contributes to vascular remodeling observed in many diseases including idiopathic pulmonary arterial hypertension. However, the role of RAS imbalance in CTEPH has not been characterized. Here, we hypothesize that RAS imbalance regulates vascular remodeling by promoting PASMC migration in CTEPH...
November 9, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
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