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https://www.readbyqxmd.com/read/29670919/dysregulated-functions-of-lung-macrophage-populations-in-copd
#1
REVIEW
Theodore S Kapellos, Kevin Bassler, Anna C Aschenbrenner, Wataru Fujii, Joachim L Schultze
Chronic obstructive pulmonary disease (COPD) is a diverse respiratory disease characterised by bronchiolitis, small airway obstruction, and emphysema. Innate immune cells play a pivotal role in the disease's progression, and in particular, lung macrophages exploit their prevalence and strategic localisation to orchestrate immune responses. To date, alveolar and interstitial resident macrophages as well as blood monocytes have been described in the lungs of patients with COPD contributing to disease pathology by changes in their functional repertoire...
2018: Journal of Immunology Research
https://www.readbyqxmd.com/read/29670467/trail-dependent-resolution-of-pulmonary-fibrosis
#2
David M Habiel, Ana Paula Moreira, Ugur B Ismailoglu, Michael P Dunleavy, Karen A Cavassani, Nico van Rooijen, Ana Lucia Coelho, Cory M Hogaboam
Idiopathic pulmonary fibrosis (IPF) is the most common form of interstitial lung disease characterized by the persistence of activated myofibroblasts resulting in excessive deposition of extracellular matrix proteins and profound tissue remodeling. In the present study, the expression of tumor necrosis factor- (TNF-) related apoptosis-inducing ligand (TRAIL) was key to the resolution of bleomycin-induced pulmonary fibrosis. Both in vivo and in vitro studies demonstrated that Gr-1+ TRAIL+ bone marrow-derived myeloid cells blocked the activation of lung myofibroblasts...
2018: Mediators of Inflammation
https://www.readbyqxmd.com/read/29655802/mars-variant-associated-with-both-recessive-interstitial-lung-and-liver-disease-and-dominant-charcot-marie-tooth-disease
#3
Jonathan Rips, Rebecca Meyer-Schuman, Oded Breuer, Reuven Tsabari, Avraham Shaag, Shoshana Revel-Vilk, Shimon Reif, Orly Elpeleg, Anthony Antonellis, Tamar Harel
Aminoacyl-tRNA synthetases (ARSs) are ubiquitously expressed enzymes responsible for charging tRNA with cognate amino acids during protein translation. Non-canonical functions are increasingly recognized, and include transcription and translation control and extracellular signaling. Monoallelic mutations in genes encoding several ARSs have been identified in axonal Charcot-Marie-Tooth (CMT2) disease, whereas biallelic mutations in ARS loci have been associated with multi-tissue syndromes, variably involving the central nervous system, lung, and liver...
April 12, 2018: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29652651/prediction-of-organ-involvement-in-systemic-sclerosis-by-serum-biomarkers-and-peripheral-endothelial-function
#4
Shin-Ya Kawashiri, Ayako Nishino, Takashi Igawa, Ayuko Takatani, Toshimasa Shimizu, Masataka Umeda, Shoichi Fukui, Akitomo Okada, Takahisa Suzuki, Tomohiro Koga, Naoki Iwamoto, Kunihiro Ichinose, Mami Tamai, Munetoshi Nakashima, Akinari Mizokami, Naoki Matsuoka, Kiyoshi Migita, Fumihide Ogawa, Satoshi Ikeda, Koji Maemura, Hideki Nakamura, Tomoki Origuchi, Takahiro Maeda, Atsushi Kawakami
OBJECTIVES: To identify prognostic factors among serum biomarkers and endothelial vasodilator function findings in patients with systemic sclerosis (SSc). METHODS: This is a clinical observational study. We assessed 60 consecutive SSc patients (44 limited cutaneous-type, 16 diffuse cutaneous-type). Circulating growth differentiation factor-15 (GDF-15), placenta growth factor (PlGF), endostatin, vascular endothelial growth factor (VEGF), and pentraxin 3 (PTX3) were measured by ELISA...
April 12, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29649237/bronchoalveolar-lavage-bal-cells-in-idiopathic-pulmonary-fibrosis-express-a-complex-pro-inflammatory-pro-repair-angiogenic-activation-pattern-likely-associated-with-macrophage-iron-accumulation
#5
Jungnam Lee, Ivan Arisi, Ermanno Puxeddu, Lazarus K Mramba, Massimo Amicosante, Carmen M Swaisgood, Marco Pallante, Mark L Brantly, C Magnus Sköld, Cesare Saltini
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease of unknown cause characterized by alveolar epithelial damage, patchy interstitial fibrosis and diffuse microvascular abnormalities. In IPF, alveolar clustering of iron-laden alveolar macrophages-a common sign of microhemorrhage, has been associated with vascular abnormalities and worsening of pulmonary hypertension. As iron-dependent ROS generation has been shown to induce unrestrained macrophage activation in disease models of vascular damage, we explored alveolar macrophage activation phenotype in IPF patients (n = 16) and healthy controls (CTR, n = 7) by RNA sequencing of bronchoalveolar lavage (BAL) cells...
2018: PloS One
https://www.readbyqxmd.com/read/29642694/integrated-quadruple-stress-echocardiography
#6
Eugenio Picano, Doralisa Morrone, Maria C Scali, Alda Huqi, Katia Coviello, Quirino Ciampi
Stress Echocardiography (SE) is an established diagnostic technique. For 40 years, the cornerstone of the technique has been the detection of regional wall motion abnormalities (RWMA), due to the underlying physiologically-relevant epicardial coronary artery stenosis. In the last decade, three new parameters (more objective than RWMA) have shown the potential to integrate and comple- ment RWMA: 1- B-lines, also known as ultrasound lung comets, as a marker of extra-vascular lung water, measured using lung ultrasound with the 4-site simplified scan symmetrically of the antero- lateral thorax on the third intercostal space, from mid-axillary to anterior axillary and mid- clavicular line; 2-left ventricular contractile reserve (LVCR), assessed as the peak stress/rest ratio of left ventricular force, also known as elastance (systolic arterial pressure by cuff sphygmomanome- ter/end-systolic volume from 2D echocardiography); 3- coronary flow velocity reserve (CFVR) on left anterior descending coronary artery, calculated as peak stress/rest ratio of diastolic peak flow velocity assessed using pulsed-wave Doppler...
April 11, 2018: Minerva Cardioangiologica
https://www.readbyqxmd.com/read/29610629/digital-clubbing-is-associated-with-higher-serum-kl-6-levels-and-lower-pulmonary-function-in-patients-with-interstitial-lung-disease
#7
Kazushige Shiraishi, Torahiko Jinta, Naoki Nishimura, Hiroshi Nakaoka, Ryosuke Tsugitomi, Kohei Okafuji, Atsushi Kitamura, Yutaka Tomishima, Gautam A Deshpande, Tomohide Tamura
Background: Although digital clubbing is a common presentation in patients with interstitial lung disease (ILD), little has been reported regarding its role in assessing patients with ILD. This study evaluated patients with ILD for the presence of clubbing and investigated its association with clinical data. Methods: We evaluated patients with ILD who visited the teaching hospital at which the study was conducted, between October 2014 and January 2015. Clubbing, evaluated using a Vernier caliper for individual patients, was defined as a phalangeal depth ratio > 1...
2018: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
https://www.readbyqxmd.com/read/29606668/rituximab-in-the-treatment-of-interstitial-lung-disease-associated-with-antisynthetase-syndrome-a-multicenter-retrospective-case-review
#8
Tracy J Doyle, Namrata Dhillon, Rachna Madan, Fernanda Cabral, Elaine A Fletcher, Diane C Koontz, Rohit Aggarwal, Juan C Osorio, Ivan O Rosas, Chester V Oddis, Paul F Dellaripa
OBJECTIVE: To assess clinical outcomes including imaging findings on computed tomography (CT), pulmonary function testing (PFT), and glucocorticoid (GC) use in patients with the antisynthetase syndrome (AS) and interstitial lung disease (ILD) treated with rituximab (RTX). METHODS: We retrospectively identified all patients at 2 institutions with AS-ILD who were treated with RTX. Baseline demographics, PFT, and chest CT were assessed before and after RTX. Two radiologists independently evaluated CT using a standardized scoring system...
April 1, 2018: Journal of Rheumatology
https://www.readbyqxmd.com/read/29605286/pulmonary-hypertension-in-patients-with-interstitial-lung-disease
#9
REVIEW
Theodoros Karampitsakos, Argyrios Tzouvelekis, Serafeim Chrysikos, Demosthenes Bouros, Iraklis Tsangaris, Wassim H Fares
Interstitial lung diseases (ILDs) comprise a broad and heterogeneous group of more than two hundred diseases with common functional characteristics. Their diagnosis and management require a multidisciplinary approach. This multidisciplinary approach involves the assessment of comorbid conditions including pulmonary hypertension (PH) that exerts a dramatic impact on survival. The current World Health Organization (WHO) classification of PH encompasses many of the interstitial lung diseases into WHO Group 3, while sarcoidosis, Pulmonary Langerhans Cell Histiocytosis and lymphangioleiomyomatosis are placed into WHO Group 5 as diseases with unclear or multifactorial mechanisms...
March 29, 2018: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29602706/baseline-lung-allograft-dysfunction-is-associated-with-impaired-survival-after-double-lung-transplantation
#10
Jonathan Liu, Kathy Jackson, Justin Weinkauf, Ali Kapasi, Alim Hirji, Steve Meyer, John Mullen, Jayan Nagendran, Dale Lien, Kieran Halloran
BACKGROUND: The prognostic value of defining normal vs abnormal baseline post-transplant lung function (or baseline lung allograft dysfunction [BLAD]) has not been studied using standardized reference values of percent predicted of the population. Our aim was to assess the association between BLAD and survival in double-lung transplant recipients and assess for potential pre-transplant donor and recipient risk factors for BLAD. METHODS: We conducted a retrospective cohort study of double-lung transplant recipients in our program during the period 2004 to 2009...
February 27, 2018: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29593714/bacterial-and-pneumocystis-infections-in-the-lungs-of-gene-knockout-rabbits-with-severe-combined-immunodeficiency
#11
Jun Song, Guoshun Wang, Mark J Hoenerhoff, Jinxue Ruan, Dongshan Yang, Jifeng Zhang, Jibing Yang, Patrick A Lester, Robert Sigler, Michael Bradley, Samantha Eckley, Kelsey Cornelius, Kong Chen, Jay K Kolls, Li Peng, Liang Ma, Yuqing Eugene Chen, Fei Sun, Jie Xu
Using the CRISPR/Cas9 gene-editing technology, we recently produced a number of rabbits with mutations in immune function genes, including FOXN1, PRKDC, RAG1, RAG2, and IL2RG. Seven founder knockout rabbits (F0) and three male IL2RG null (-/y) F1 animals demonstrated severe combined immunodeficiency (SCID), characterized by absence or pronounced hypoplasia of the thymus and splenic white pulp, and absence of immature and mature T and B-lymphocytes in peripheral blood. Complete blood count analysis showed severe leukopenia and lymphocytopenia accompanied by severe neutrophilia...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29588624/stereotactic-body-radiation-therapy-sbrt-in-the-management-of-non-small-cell-lung-cancer-clinical-impact-and-patient-perspectives
#12
REVIEW
Elysia K Donovan, Anand Swaminath
Stereotactic body radiation therapy (SBRT) has emerged as a new technology in radiotherapy delivery, allowing for potentially curative treatment in many patients previously felt not to be candidates for radical surgical resection of stage I non-small-cell lung cancer (NSCLC). Several studies have demonstrated very high local control rates using SBRT, and more recent data have suggested overall survival may approach that of surgery in operable patients. However, SBRT is not without unique toxicities, and the balance of toxicity, and effect on patient-reported quality of life need to be considered with respect to oncologic outcomes...
2018: Lung Cancer: Targets and Therapy
https://www.readbyqxmd.com/read/29587761/evaluation-of-oxidative-stress-biomarkers-in-idiopathic-pulmonary-fibrosis-and-therapeutic-applications-a-systematic-review
#13
REVIEW
Alessandro G Fois, Panagiotis Paliogiannis, Salvatore Sotgia, Arduino A Mangoni, Elisabetta Zinellu, Pietro Pirina, Ciriaco Carru, Angelo Zinellu
INTRODUCTION: Idiopathic pulmonary fibrosis (IPF), a fatal lung disease of unknown origin, is characterized by chronic and progressive fibrosing interstitial pneumonia which progressively impairs lung function. Oxidative stress is one of the main pathogenic pathways in IPF. The aim of this systematic review was to describe the type of markers of oxidative stress identified in different biological specimens and the effects of antioxidant therapies in patients with IPF. METHODS: We conducted a systematic search of publications listed in electronic databases (Pubmed, Web of Science, Scopus and Google Scholar) from inception to October 2017...
March 27, 2018: Respiratory Research
https://www.readbyqxmd.com/read/29587263/a-real-life-multicenter-national-study-on-nintedanib-in-severe-idiopathic-pulmonary-fibrosis
#14
Sergio Harari, Antonella Caminati, Venerino Poletti, Marco Confalonieri, Stefano Gasparini, Donato Lacedonia, Fabrizio Luppi, Alberto Pesci, Alfredo Sebastiani, Paolo Spagnolo, Carlo Vancheri, Elisabetta Balestro, Martina Bonifazi, Stefania Cerri, Federica De Giacomi, Rossana Della Porta, Maria Pia Foschino Barbaro, Annalisa Fui, Patrizio Pasquinelli, Roberta Rosso, Sara Tomassetti, Claudia Specchia, Paola Rottoli
BACKGROUND: Two therapeutic options are currently available for patients with mild-to-moderate idiopathic pulmonary fibrosis (IPF): pirfenidone and nintedanib. To date, there is still insufficient data on the efficacy of these 2 agents in patients with more severe disease. OBJECTIVES: This national, multicenter, retrospective real-life study was intended to determine the impact of nintedanib on the treatment of patients with severe IPF. METHODS: All patients included had severe IPF and had to have at least 6 months of follow-up before and at least 6 months of follow-up after starting nintedanib...
March 27, 2018: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/29575410/performance-of-the-st-george-s-respiratory-questionnaire-in-patients-with-connective-tissue-disease-associated-interstitial-lung-disease
#15
Atsushi Suzuki, Yasuhiro Kondoh, Jeffrey J Swigris, Masahiko Ando, Tomoki Kimura, Kensuke Kataoka, Yasuhiko Yamano, Taiki Furukawa, Mari Numata, Koji Sakamoto, Yoshinori Hasegawa
BACKGROUND AND OBJECTIVE: The St George's Respiratory Questionnaire (SGRQ) is a self-administered questionnaire used to assess health-related quality of life (HRQoL) in various chronic respiratory diseases. Few studies have assessed the performance of the SGRQ in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). We aimed to examine the SGRQ's performance characteristics and generate data to support its reliability and validity in patients with CTD-ILD...
March 25, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29566745/antibodies-against-chemokine-receptors-cxcr3-and-cxcr4-predict-progressive-deterioration-of-lung-function-in-patients-with-systemic-sclerosis
#16
Florian Weigold, Jeannine Günther, Moritz Pfeiffenberger, Otavio Cabral-Marques, Elise Siegert, Duska Dragun, Aurélie Philippe, Ann-Katrin Regensburger, Andreas Recke, Xinhua Yu, Frank Petersen, Rusan Catar, Robert Biesen, Falk Hiepe, Gerd R Burmester, Harald Heidecke, Gabriela Riemekasten
BACKGROUND: The chemokine receptors CXCR3 and CXCR4 are involved in the pathogenesis of fibrosis, a key feature of systemic sclerosis (SSc). It is hypothesized that immunoglobulin (Ig)G antibodies (abs) against these two receptors are present in patients with SSc and are associated with clinical findings. METHODS: Anti-CXCR3 and anti-CXCR4 ab levels were measured in 449 sera from 327 SSc patients and in 234 sera from healthy donors (HD) by enzyme-linked immunosorbent assay (ELISA)...
March 22, 2018: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29566461/surfactant-deficiency-syndrome-in-an-infant-with-a-c-terminal-frame-shift-in-abca3-a-case-report
#17
Nour Akil, Anthony J Fischer
Deficiency in ATP binding cassette A3 (ABCA3) causes neonatal respiratory distress, hypoxemic respiratory failure, and interstitial lung disease. ABCA3 transports phospholipids into the lamellar bodies of type II alveolar cells, a critical step in alveolar surfactant production. We report a term infant with ABCA3 surfactant deficiency syndrome with the E292V (c.875A>T; p.Glu292Val) mutation in trans with a novel C-terminal frame shift mutation (c.4938delC; p.Met1647fs). This mutation removes the final 58 amino acids and substitutes 33 incorrect amino acids...
March 22, 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29555917/comparison-of-cpi-and-gap-models-in-patients-with-idiopathic-pulmonary-fibrosis-a-nationwide-cohort-study
#18
Sang Hoon Lee, Jong Sun Park, Song Yee Kim, Dong Soon Kim, Young Whan Kim, Man Pyo Chung, Soo Taek Uh, Choon Sik Park, Sung Woo Park, Sung Hwan Jeong, Yong Bum Park, Hong Lyeol Lee, Jong Wook Shin, Eun Joo Lee, Jin Hwa Lee, Yangin Jegal, Hyun Kyung Lee, Yong Hyun Kim, Jin Woo Song, Moo Suk Park
The clinical course of idiopathic pulmonary fibrosis (IPF) is difficult to predict, partly owing to its heterogeneity. Composite physiologic index (CPI) and gender-age-physiology (GAP) models are easy-to-use predictors of IPF progression. This study aimed to compare the predictive values of these two models. From 2003 to 2007, the Korean Interstitial Lung Disease (ILD) Study Group surveyed ILD patients using the 2002 ATS/ERS criteria. A total of 832 patients with IPF were enrolled in this study. CPI was calculated as follows: 91...
March 19, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29543042/nintedanib-reduces-pulmonary-fibrosis-in-a-model-of-rheumatoid-arthritis-associated-interstitial-lung-disease
#19
Elizabeth F Redente, Martin A Aguilar, Bart P Black, Benjamin Edelman, Ali Bahadur, Stephen M Humphries, David A Lynch, Lutz Wollin, David W H Riches
Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) develops in approximately 20% of patients with RA. SKG mice, which are genetically prone to developing autoimmune arthritis, develop a pulmonary interstitial pneumonia that resembles human cellular and fibrotic non-specific interstitial pneumonia. Nintedanib, a tyrosine kinase inhibitor approved for the treatment of idiopathic pulmonary fibrosis, has been shown to reduce lung function decline. Therefore, we investigated the effect of nintedanib on the development of pulmonary fibrosis and joint disease in female SKG mice with arthritis induced by intraperitoneal injection of zymosan (5 mg)...
March 15, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29535639/vascular-endothelial-cell-specific-connective-tissue-growth-factor-ctgf-is-necessary-for-development-of-chronic-hypoxia-induced-pulmonary-hypertension
#20
Liya Pi, Chunhua Fu, Yuanquing Lu, Junmei Zhou, Marda Jorgensen, Vinayak Shenoy, Kenneth E Lipson, Edward W Scott, Andrew J Bryant
Chronic hypoxia frequently complicates the care of patients with interstitial lung disease, contributing to the development of pulmonary hypertension (PH), and premature death. Connective tissue growth factor (CTGF), a matricellular protein of the Cyr61/CTGF/Nov (CCN) family, is known to exacerbate vascular remodeling within the lung. We have previously demonstrated that vascular endothelial-cell specific down-regulation of CTGF is associated with protection against the development of PH associated with hypoxia, though the mechanism for this effect is unknown...
2018: Frontiers in Physiology
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