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https://www.readbyqxmd.com/read/28096284/pulmonary-vascular-and-cardiac-impairment-in-interstitial-lung-disease
#1
REVIEW
Marios Panagiotou, Alistair C Church, Martin K Johnson, Andrew J Peacock
Pulmonary vascular and cardiac impairment is increasingly appreciated as a major adverse factor in the natural history of interstitial lung disease. This clinically orientated review focuses on the current concepts in the pathogenesis, pathophysiology and implications of the detrimental sequence of increased pulmonary vascular resistance, pre-capillary pulmonary hypertension and right heart failure in interstitial lung disease, and provides guidance on its management.
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28093201/molecular-targets-of-the-warburg-effect-and-inflammatory-cytokines-in-the-pathogenesis-of-pulmonary-artery-hypertension
#2
REVIEW
Na Liu, Stephanie Parry, Yunbin Xiao, Shenghua Zhou, Qiming Liu
Pulmonary arterial hypertension (PAH) is a progressive vascular disease characterized by increased pulmonary arterial pressure and vasoconstriction and structural remolding of pulmonary arterioles. Recent clinical and experimental studies have discovered the relationship between metabolic alterations and the pathogenesis of PAH. The primary metabolic alteration, previously demonstrated in various cancers, is a gradual change in energy generated from complete aerobic cellular respiration to from solely "aerobic glycolysis," termed the "Warburg effect...
January 13, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28090303/genotype-phenotype-effects-of-bmpr2-mutations-on-disease-severity-in-mouse-models-of-pulmonary-hypertension
#3
Andrea L Frump, Arunima Datta, Sampa Ghose, James West, Mark P de Caestecker
More than 350 mutations in the type-2 BMP (bone morphogenetic protein) receptor, BMPR2, have been identified in patients with heritable pulmonary arterial hypertension (HPAH). However, only 30% of BMPR2 mutation carriers develop PAH, and we cannot predict which of these carriers will develop clinical disease. One possibility is that the nature of the BMPR2 mutation affects disease severity. This hypothesis has been difficult to test clinically, given the rarity of HPAH and the complexity of the confounding genetic and environmental risk factors...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090302/three-dimensional-micro-computed-tomography-analysis-of-the-lung-vasculature-and-differential-adipose-proteomics-in-the-sugen-hypoxia-rat-model-of-pulmonary-arterial-hypertension
#4
Kelly J Shields, Kostas Verdelis, Michael J Passineau, Erin M Faight, Lee Zourelias, Changgong Wu, Rong Chong, Raymond L Benza
Pulmonary arterial hypertension (PAH) is a rare disease characterized by significant vascular remodeling. The obesity epidemic has produced great interest in the relationship between small visceral adipose tissue depots producing localized inflammatory conditions, which may link metabolism, innate immunity, and vascular remodeling. This study used novel micro computed tomography (microCT) three-dimensional modeling to investigate the degree of remodeling of the lung vasculature and differential proteomics to determine small visceral adipose dysfunction in rats with severe PAH...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090301/pulmonary-pulse-wave-transit-time-is-associated-with-right-ventricular-pulmonary-artery-coupling-in-pulmonary-arterial-hypertension
#5
Kurt W Prins, E Kenneth Weir, Stephen L Archer, Jeremy Markowitz, Lauren Rose, Marc Pritzker, Richard Madlon-Kay, Thenappan Thenappan
Pulmonary pulse wave transit time (pPTT), defined as the time for the systolic pressure pulse wave to travel from the pulmonary valve to the pulmonary veins, has been reported to be reduced in pulmonary arterial hypertension (PAH); however, the underlying mechanism of reduced pPTT is unknown. Here, we investigate the hypothesis that abbreviated pPTT in PAH results from impaired right ventricular-pulmonary artery (RV-PA) coupling. We quantified pPTT using pulsed-wave Doppler ultrasound from 10 healthy age- and sex-matched controls and 36 patients with PAH...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090294/the-effects-of-pulmonary-vasodilating-agents-on-right-ventricular-parameters-in-severe-group-3-pulmonary-hypertension-a-pilot-study
#6
Takahiro Sato, Ichizo Tsujino, Ayako Sugimoto, Toshitaka Nakaya, Taku Watanabe, Hiroshi Ohira, Masaru Suzuki, Satoshi Konno, Noriko Oyama-Manabe, Masaharu Nishimura
Pulmonary arterial hypertension (PAH)-approved vasodilators improve right ventricular (RV) function in patients with PAH. However, whether PAH-approved drugs ameliorate RV morphology and function in lung disease-associated pulmonary hypertension (lung-PH) remains unclear. We aimed to prospectively evaluate the changes in RV volume and ejection fraction (RVEF) in 14 consecutive severe lung-PH patients treated with PAH-approved vasodilators. Severe lung-PH was defined as a mean pulmonary arterial pressure (MPAP) of ≥35 mmHg or an MPAP of ≥25 mmHg with a cardiac index (L/min/m(2)) of <2...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090291/macrophage-migration-inhibitory-factor-as-a-novel-biomarker-of-portopulmonary-hypertension
#7
Hilary M DuBrock, Josanna M Rodriguez-Lopez, Barbara L LeVarge, Michael P Curry, Paul A VanderLaan, Zsuzsanna K Zsengeller, Elizabeth Pernicone, Ioana R Preston, Paul B Yu, Ivana Nikolic, Dihua Xu, Ravi I Thadhani, Richard N Channick, S Ananth Karumanchi
Portopulmonary hypertension (POPH) is a poorly understood complication of liver disease associated with significant morbidity and mortality. We sought to identify novel biomarkers of POPH disease presence and severity. We performed a prospective, multicenter, case-control study involving patients with liver disease undergoing right heart catheterization. POPH cases were defined as a mean pulmonary arterial pressure (mPAP) ≥25 mmHg and pulmonary vascular resistance (PVR) >240 dynes˙s˙cm(-5). Plasma samples were collected from the systemic and pulmonary circulation, and antibody microarray was used to identify biomarkers...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090290/shared-gene-expression-patterns-in-mesenchymal-progenitors-derived-from-lung-and-epidermis-in-pulmonary-arterial-hypertension-identifying-key-pathways-in-pulmonary-vascular-disease
#8
Christa Gaskill, Shennea Marriott, Sidd Pratap, Swapna Menon, Lora K Hedges, Joshua P Fessel, Jonathan A Kropski, DeWayne Ames, Lisa Wheeler, James E Loyd, Anna R Hemnes, Dennis R Roop, Dwight J Klemm, Eric D Austin, Susan M Majka
Rapid access to lung-derived cells from stable subjects is a major challenge in the pulmonary hypertension field, given the relative contraindication of lung biopsy. In these studies, we sought to demonstrate the importance of evaluating a cell type that actively participates in disease processes, as well as the potential to translate these findings to vascular beds in other nonlung tissues, in this instance perivascular skin mesenchymal cells (MCs). We utilized posttransplant or autopsy lung explant-derived cells (ABCG2-expressing mesenchymal progenitor cells [MPCs], fibroblasts) and skin-derived MCs to test the hypothesis that perivascular ABCG2 MPCs derived from pulmonary arterial hypertension (PAH) patient lung and skin would express a gene profile reflective of ongoing vascular dysfunction...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090288/expression-profiling-elucidates-a-molecular-gene-signature-for-pulmonary-hypertension-in-sarcoidosis
#9
Sunit Singla, Tong Zhou, Kamran Javaid, Taimur Abbasi, Nancy Casanova, Wei Zhang, Shwu-Fan Ma, Michael S Wade, Imre Noth, Nadera J Sweiss, Joe G N Garcia, Roberto F Machado
Pulmonary hypertension (PH), when it complicates sarcoidosis, carries a poor prognosis, in part because it is difficult to detect early in patients with worsening respiratory symptoms. Pathogenesis of sarcoidosis occurs via incompletely characterized mechanisms that are distinct from the mechanisms of pulmonary vascular remodeling well known to occur in conjunction with other chronic lung diseases. To address the need for a biomarker to aid in early detection as well as the gap in knowledge regarding the mechanisms of PH in sarcoidosis, we used genome-wide peripheral blood gene expression analysis and identified an 18-gene signature capable of distinguishing sarcoidosis patients with PH (n = 8), sarcoidosis patients without PH (n = 17), and healthy controls (n = 45)...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090287/transcription-factors-transcriptional-coregulators-and-epigenetic-modulation-in-the-control-of-pulmonary-vascular-cell-phenotype-therapeutic-implications-for-pulmonary-hypertension-2015-grover-conference-series
#10
REVIEW
Soni S Pullamsetti, Frédéric Perros, Prakash Chelladurai, Jason Yuan, Kurt Stenmark
Pulmonary hypertension (PH) is a complex and multifactorial disease involving genetic, epigenetic, and environmental factors. Numerous stimuli and pathological conditions facilitate severe vascular remodeling in PH by activation of a complex cascade of signaling pathways involving vascular cell proliferation, differentiation, and inflammation. Multiple signaling cascades modulate the activity of certain sequence-specific DNA-binding transcription factors (TFs) and coregulators that are critical for the transcriptional regulation of gene expression that facilitates PH-associated vascular cell phenotypes, as demonstrated by several studies summarized in this review...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090286/novel-methods-in-pulmonary-hypertension-phenotyping-in-the-age-of-precision-medicine-2015-grover-conference-series
#11
REVIEW
Jarrod W Barnes, Adriano R Tonelli, Gustavo A Heresi, Jennie E Newman, Noël E Mellor, David E Grove, Raed A Dweik
Among pulmonary vascular diseases, pulmonary hypertension (PH) is the best studied and has been the focus of our work. The current classification of PH is based on a relatively simple combination of patient characteristics and hemodynamics. This leads to inherent limitations, including the inability to customize treatment and the lack of clarity from a more granular identification based on individual patient phenotypes. Accurate phenotyping of PH can be used in the clinic to select therapies and determine prognosis and in research to increase the homogeneity of study cohorts...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090285/pulmonary-vascular-and-ventricular-dysfunction-in-the-susceptible-patient-2015-grover-conference-series
#12
REVIEW
Bradley A Maron, Roberto F Machado, Larissa Shimoda
Pulmonary blood vessel structure and tone are maintained by a complex interplay between endogenous vasoactive factors and oxygen-sensing intermediaries. Under physiological conditions, these signaling networks function as an adaptive interface between the pulmonary circulation and environmental or acquired perturbations to preserve oxygenation and maintain systemic delivery of oxygen-rich hemoglobin. Chronic exposure to hypoxia, however, triggers a range of pathogenetic mechanisms that include hypoxia-inducible factor 1α (HIF-1α)-dependent upregulation of the vasoconstrictor peptide endothelin 1 in pulmonary endothelial cells...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28089564/cardiorespiratory-fitness-after-transient-ischemic-attack-and-minor-ischemic-stroke-baseline-data-of-the-moveit-study
#13
H Myrthe Boss, IngerA Deijle, SanderM Van Schaik, EdwinC de Melker, BobT J van den Berg, HenryC Weinstein, MirjamI Geerlings, L Jaap Kappelle, RenskeM Van den Berg-Vos
BACKGROUND: Cardiorespiratory fitness (CRF) is reduced in patients with stroke. It is unclear whether it is also reduced in patients with a transient ischemic attack (TIA) or minor stroke. We investigated the CRF in patients with a recent TIA or minor stroke and explored which determinants are associated with a lower fitness. METHODS: In 113 patients with a recent TIA or minor ischemic stroke (64 (SD = 10) years of age; 49 (IQR 27-86) days post TIA or stroke), the peak oxygen consumption (VO2peak) was determined in a symptom-limited ramp exercise test...
January 12, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28089339/discovery-of-a-murine-model-of-clinical-pah-mission-impossible
#14
REVIEW
Zhiyu Dai, You-Yang Zhao
Pulmonary arterial hypertension (PAH) is a lung vascular disease characterized with a progressive increase of pulmonary vascular resistance and obliterative pulmonary vascular remodeling resulting in right heart failure and premature death. In this brief review, we document the recent advances in identifying genetically modified murine models of PH, with a focus on the recent discovery of the mouse model of Tie2 Cre-mediated deletion of prolyl hydroxylase 2, which exhibits progressive obliterative vascular remodeling, severe PAH, and right heart failure, thus recapitulating many of the features of clinical PAH...
December 15, 2016: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28088960/-role-and-mechanism-of-hydrogen-sulfide-in-cigarette-smoke-induced-chronic-obstructive-pulmonary-disease-related-pulmonary-vascular-remodeling-in-rats
#15
M X Li, Y H Chen, C C Liao, F Lin, Y Bai, W J Mi, Y Sun, Y F Qi
Objective: To investigate the role and mechanism of hydrogen sulfide (H2S) in rats with chronic obstructive pulmonary disease (COPD) related pulmonary vascular remodeling. Methods: Twenty four healthy male Sprague-Dawley rats were randomly divided into 4 groups: control group, cigarette smoke (CS) group, CS+ Sodium hydrosulfide (NaHS) group and CS+ DL-propargylglycine (PPG) group. Rats in control group were fed normally and breathed clear air, and for the rest groups, passive cigarette smoke inhalation method were adopted to establish COPD model...
January 10, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28088638/the-dlno-dlco-ratio-physiological-significance-and-clinical-implications
#16
J M B Hughes, A T Dinh-Xuan
DLNO/DLCO directly measures the ratio of the diffusing capacities of the lung for nitric oxide (NO) and carbon monoxide (CO). In terms of the Roughton and Forster, 1957; equation, 1/DL=1/Dm+1/θVc, where Dm is the membrane (Dm) and θVc is the red cell component of the overall diffusing conductance (DL), DLNO mostly reflects the Dm component and DLCO the θVc red cell component. The DLNO/DLCO ratio is positively related to the DmCO/Vc ratio and the CO red cell resistance (1/θCOVc) as a percentage of the total resistance (1/DLCO), independent of the absolute values of DLNO or DLCO...
January 11, 2017: Respiratory Physiology & Neurobiology
https://www.readbyqxmd.com/read/28087752/deficient-retinoid-driven-angiogenesis-may-contribute-to-failure-of-adult-human-lung-regeneration-in-emphysema
#17
John-Poul Ng-Blichfeldt, Joana Alçada, M Angeles Montero, Charlotte H Dean, Uta Griesenbach, Mark J Griffiths, Matthew Hind
BACKGROUND: Molecular pathways that regulate alveolar development and adult repair represent potential therapeutic targets for emphysema. Signalling via retinoic acid (RA), derived from vitamin A, is required for mammalian alveologenesis, and exogenous RA can induce alveolar regeneration in rodents. Little is known about RA signalling in the human lung and its potential role in lung disease. OBJECTIVES: To examine regulation of human alveolar epithelial and endothelial repair by RA, and characterise RA signalling in human emphysema...
January 13, 2017: Thorax
https://www.readbyqxmd.com/read/28079844/serum-interleukin-6-in-systemic-sclerosis-and-its-correlation-with-disease-parameters-and-cardiopulmonary-involvement
#18
Rasha A Abdel-Magied, Shereen R Kamel, Azza Farag Said, Hazem M Ali, Ehab A Abdel Gawad, Mahmoud M Moussa
OBJECTIVE: To assess serum interleukin-6 (IL-6)level in patients with systemic sclerosis (SSc) and its correlations with European Scleroderma Study Group activity score (EUSTAR), Scleroderma Assessment Questionnaire (SAQ), disability index and cardiopulmonary involvement. METHODS: Twenty SSc patients and 10 matched healthy controls were included. Serum IL-6 was measured in patients and controls. Disease activity, status,and disability were assessed.Cardiopulmonary involvement was evaluated by pulmonary function tests (PFTs), six minute walk test, echocardiography, and high resolution computed tomography (HRCT) of chest...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28077433/osteoprotegerin-disruption-attenuates-hysu-induced-pulmonary-hypertension-through-integrin-%C3%AE-v%C3%AE-3-fak-akt-pathway-suppression
#19
Daile Jia, Qian Zhu, Huan Liu, Caojian Zuo, Yuhu He, Guilin Chen, Ankang Lu
BACKGROUND: Pulmonary arterial remodeling characterized by increased vascular smooth muscle proliferation is commonly seen in life-threatening disease, pulmonary arterial hypertension (PAH). Clinical studies have suggested a correlation between osteoprotegerin serum levels and PAH severity. Here, we aimed to invhestigate vascular osteoprotegerin expression and its effects on pulmonary arterial smooth muscle cell proliferation in vitro and in vivo, as well as examine the signal transduction pathways mediating its activity...
February 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28070482/pulmonary-veno-occlusive-disease-two-children-with-gradual-disease-progression
#20
Ronald W Day, Parker W Clement, Aimee O Hersh, Susan M Connors, Kelli L Sumner, D Hunter Best, Mouied Alashari
Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis are rare forms of pulmonary vascular disease. We report two cases of affected children who had evidence of pulmonary hypertension 3-5 years before developing radiographic findings of pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis. Both patients experienced a moderate decrease in pulmonary arterial pressure during acute vasodilator testing. Both patients experienced an improvement in six-minute walk performance without an increase in pulmonary edema when treated with targeted therapy for pulmonary hypertension...
2017: Respiratory Medicine Case Reports
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