Abstract:
: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. By analyzing the genetic signatures and pathways associated with abnormal ABCG2 lung MPC phenotypes during PAH and evaluating them in lung- and skin-derived MCs, we have identified potential predictor genes for detection of PAH as well as a targetable mechanism to restore MPCs and microvascular function. These studies are the first to explore the utility of expanding the study of ABCG2 MPC regulation of the pulmonary microvasculature to the epidermis, in order to identify potential markers for adult lung vascular disease, such as PAH.
journal_name
Pulm Circjournal_title
Pulmonary circulationauthors
Gaskill C,Marriott S,Pratap S,Menon S,Hedges LK,Fessel JP,Kropski JA,Ames D,Wheeler L,Loyd JE,Hemnes AR,Roop DR,Klemm DJ,Austin ED,Majka SMdoi
10.1086/688314subject
Has Abstractpub_date
2016-12-01 00:00:00pages
483-497issue
4eissn
2045-8932issn
2045-8940pii
PC2013372journal_volume
6pub_type
杂志文章abstract::N-terminal pro B-type natriuretic peptide (NT-proBNP) is a product of cleavage of the cardiac prohormone pro B-type natriuretic peptide into its active form. It has proven to be a useful biomarker in left heart failure. However, studies examining the utility of serial measurements of NT-proBNP in pulmonary arterial hy...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.83450
更新日期:2011-04-01 00:00:00
abstract::Levels of the cAMP-responsive transcription factor, CREB, are reduced in medial smooth muscle cells in remodeled pulmonary arteries from hypertensive calves and rats with chronic hypoxia-induced pulmonary hypertension. Here, we show that chronic hypoxia fails to promote CREB depletion in pulmonary artery smooth muscle...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019898374
更新日期:2020-04-14 00:00:00
abstract::Pulmonary arterial dilatation associated with pulmonary hypertension may result in significant compression of local structures. Left main coronary artery and left recurrent laryngeal nerve compression have been described. Tracheobronchial compression from pulmonary arterial dilatation is rare in adults, and there are ...
journal_title:Pulmonary circulation
pub_type:
doi:10.1086/683687
更新日期:2015-12-01 00:00:00
abstract::Right ventricular failure (RVF) is the most important prognostic factor for both morbidity and mortality in pulmonary arterial hypertension (PAH), but also occurs in numerous other common diseases and conditions, including left ventricle dysfunction. RVF remains understudied compared with left ventricular failure (LVF...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217714463
更新日期:2017-07-01 00:00:00
abstract::The biological mechanisms behind the development of pulmonary hypertension in the setting of left heart failure (HF-PH), including combined pre- and post-capillary pulmonary hypertension (Cpc-PH), remains unclear. This study aimed to use candidate polymorphisms in nitric oxide synthase (NOS) genes to explore the role ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018773049
更新日期:2018-04-01 00:00:00
abstract::Increased peripheral resistance of small distal pulmonary arteries is a hallmark signature of pulmonary hypertension (PH) and is believed to be the consequence of enhanced vasoconstriction to agonists, thickening of the arterial wall due to remodeling, and increased thrombosis. The elevation in arterial tone in PH is ...
journal_title:Pulmonary circulation
pub_type: 杂志文章,评审
doi:10.1086/680189
更新日期:2015-06-01 00:00:00
abstract::African Americans (AA) have a higher incidence of pulmonary hypertension (PH) risk factors. Few studies have examined the racial differences in the prevalence and etiology of PH and direct comparison of invasive hemodynamics between AAs and Caucasians has rarely been reported. In this study, we examined whether racial...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018764273
更新日期:2018-04-01 00:00:00
abstract::Retinal vessel diameters have been associated with left ventricular morphology and function but their relationship with the right ventricle (RV) has not been studied. We hypothesized that wider retinal venules and narrower retinal arterioles are associated with RV morphology and function. RV end-diastolic mass (RVEDM)...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018819781
更新日期:2018-12-03 00:00:00
abstract::Pulmonary hypertension is a multifactorial disease with a high morbidity and mortality. Right ventricular function is the most important predictor of morbidity and mortality in patients suffering from pulmonary hypertension, but currently there are no approved treatments directly supporting the failing right ventricle...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018790905
更新日期:2018-07-01 00:00:00
abstract::Inhaled iloprost is an effective therapy for patients with pulmonary arterial hypertension (PAH); however, some patients experience extended inhalation times when using the V10 formulation (10.0 µg/mL) to deliver a 5 -µg dose (at mouthpiece) and are at risk of incomplete inhalations and reduced inhalation frequency. V...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018798921
更新日期:2018-10-01 00:00:00
abstract::Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of unresolved organised pulmonary emboli/thrombi obstructing the major pulmonary arteries. The aim of this study was to estimate the incidence and risk factors of CTEPH in a cohort with first venous thromboembolism (VTE). This was a population-bas...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018791358
更新日期:2018-07-01 00:00:00
abstract::Optimal pulmonary hypertension (PH) management relies on a timely, accurate diagnosis and follow-up in specialized clinics by multidisciplinary teams that have clearly defined responsibilities and protocols. Internationally agreed criteria for expert center staff are lacking, particularly with respect to nurses, who o...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019855611
更新日期:2019-04-01 00:00:00
abstract::Inhaled treprostinil (Tyvaso) has been shown to be a safe and effective addition to pulmonary arterial hypertension (PAH) oral therapies; however, the respiratory-related safety profile of inhaled treprostinil required further elucidation in the setting of routine clinical care. The objectives of this study were to ch...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/688059
更新日期:2016-09-01 00:00:00
abstract::Low-grade albuminuria, determined by the urinary albumin to creatinine ratio, has been linked to systemic vascular dysfunction and is associated with cardiovascular mortality. Pulmonary arterial hypertension is related to mutations in the bone morphogenetic protein receptor type 2, pulmonary vascular dysfunction and i...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018824564
更新日期:2019-04-01 00:00:00
abstract::We hypothesized that vibrations created by the pulmonary circulation would create sound like the vocal cords during speech and that subjects with pulmonary artery hypertension (PAH) might have a unique sound signature. We recorded heart sounds at the cardiac apex and the second left intercostal space (2LICS), using a ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/683694
更新日期:2015-12-01 00:00:00
abstract::South America is a territory of 17,819,100 km(2), where ∼388 million people live in 13 countries. In the region, access to medical assistance (e.g., for treatment of cardiovascular disorders) is relatively easy in metropolitan areas but difficult in remote places such as the Andes and the Amazon. Altitudes up to ∼6,70...
journal_title:Pulmonary circulation
pub_type: 杂志文章,评审
doi:10.1086/676747
更新日期:2014-09-01 00:00:00
abstract::Pulmonary hypertension is recognized as a leading cause of morbidity and mortality in patients with sickle cell disease (SCD). We now report benchtop phenotyping from the explanted lungs of the first successful lung transplant in SCD. Pulmonary artery smooth muscle cells (PASMCs) cultured from the explanted lungs were...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/674754
更新日期:2013-12-01 00:00:00
abstract::Obesity is causally linked to a number of comorbidities, including cardiovascular disease, diabetes, renal dysfunction, and cancer. Obesity has also been linked to pulmonary disorders, including pulmonary arterial hypertension (PAH). It was long believed that obesity-related PAH was the result of hypoventilation and h...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/678510
更新日期:2014-12-01 00:00:00
abstract::Inappropriate mechanical ventilation may induce hemodynamic alterations through cardiopulmonary interactions. The aim of this study was to explore the relationship between airway pressure and central venous pressure during the first 72 h of mechanical ventilation and its relevance to patient outcomes. We conducted a r...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020970363
更新日期:2020-11-25 00:00:00
abstract::Pulmonary arterial hypertension (PAH) is a major complication of sickle cell disease (SCD). Low levels of apolipoprotein A1 (Apo-A1) have been implicated in the development of PAH in SCD. We speculate that lower levels of Apo-A1 are related to dysregulation of the ubiquitin-proteasome pathway (UPP). Of 36 recruited pa...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/674763
更新日期:2013-12-01 00:00:00
abstract::The present study aimed to propose the pulmonary hypertension for predicting left ventricular dysfunction in adults after patent ductus arteriosus closure. A total of 183 patients (age ≥18 years) after patent ductus arteriosus occlusion were retrospectively collected in this study. In brief, pre-, post-procedure and s...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019888428
更新日期:2019-11-15 00:00:00
abstract::Pulmonary arterial hypertension (PAH) patients have distinct disease courses and responses to treatment, but current diagnostic and treatment schemes provide limited insight. We aimed to see if cluster analysis could distinguish clinical phenotypes in PAH. An unbiased cluster analysis was performed on 17 baseline clin...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217705891
更新日期:2017-04-01 00:00:00
abstract::Right ventricular (RV) afterload consists of both resistive and capacitive (pulsatile) components. Total afterload can be measured directly with pulmonary artery input impedance spectra or estimated, either with lumped-parameter modeling or by pressure-volume analysis. However, the inverse, hyperbolic relationship bet...
journal_title:Pulmonary circulation
pub_type: 杂志文章,评审
doi:10.1086/676020
更新日期:2014-06-01 00:00:00
abstract::There are few data on the epidemiology of pulmonary hypertension (PH)-related hospitalizations in children in the United States. Our aim was to determine hospital mortality, length of hospitalization, and hospital charges pertaining to PH-related hospitalizations and also the effects of codiagnoses and comorbidities. ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/681226
更新日期:2015-06-01 00:00:00
abstract::Right-ventricular function is a good indicator of pulmonary arterial hypertension (PAH) prognosis; however, how the right ventricle (RV) adapts to the pressure overload is not well understood. Here, we aimed at characterizing the time course of RV early remodeling and discriminate the contribution of ventricular geome...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018800439
更新日期:2018-10-01 00:00:00
abstract::Adults with sickle cell disease can develop pulmonary hypertension from a multitude of etiologies. Classified as WHO Group 5, there are no therapies approved for the treatment of sickle cell disease-pulmonary hypertension. Thromboembolic disease is prevalent in sickle cell disease and can lead to pulmonary hypertensio...
journal_title:Pulmonary circulation
pub_type:
doi:10.1177/2045894018791802
更新日期:2018-10-01 00:00:00
abstract::Infection, thrombosis, and catheter dislodgment are well-recognized potential complications of chronic intravenous prostanoid therapy for pulmonary arterial hypertension. As long-term outcomes of pulmonary hypertension patients improve, novel adverse events are likely to arise. We describe the sudden development of un...
journal_title:Pulmonary circulation
pub_type:
doi:10.1177/2045893217748054
更新日期:2018-01-01 00:00:00
abstract::The aim of this study was to investigate whether a dual endothelin receptor antagonist bosentan modulates the kinetics of bone marrow-derived stem cells in inhibiting the development of pulmonary hypertension. Bone marrow chimeric mice, transplanted with enhanced green fluorescent protein (eGFP)-positive bone marrow m...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020919355
更新日期:2020-05-14 00:00:00
abstract::Patients with pulmonary hypertension are more sedentary than the general population, but attitudes and experiences that may influence their exercise behaviour remain poorly understood. This study identified patterns of behaviour, attitudes towards exercise, barriers and enablers of exercise for people living with pulm...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020968023
更新日期:2020-11-16 00:00:00
abstract::Endothelial dysfunction plays an important role in the pathogenesis of pulmonary arterial hypertension (PAH) in sickle cell disease (SCD). A variety of evidence suggests that circulating endothelial progenitor cells (EPCs) play an integral role in vascular repair. We hypothesized that SCD patients with PAH are deficie...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.94834
更新日期:2012-01-01 00:00:00