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Find video protocols related to scientific articles indexed in Pubmed.
Experimental validation of the hyperpolarized (129) Xe chemical shift saturation recovery technique in healthy volunteers and subjects with interstitial lung disease.
Magn Reson Med
PUBLISHED: 08-08-2014
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To assess the sensitivity of the hyperpolarized (129) Xe chemical shift saturation recovery (CSSR) technique for noninvasive quantification of changes to lung microstructure and function in idiopathic pulmonary fibrosis (IPF) and systemic sclerosis (SSc).
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Dynamic contrast-enhanced magnetic resonance imaging in patients with pulmonary arterial hypertension.
Pulm Circ
PUBLISHED: 07-10-2014
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Dynamic contrast-enhanced (DCE) time-resolved magnetic resonance (MR) imaging is a technique whereby the passage of an intravenous contrast bolus can be tracked through the pulmonary vascular system. The aim of this study was to investigate the prognostic significance of DCE-MR pulmonary blood transit times in patients with pulmonary arterial hypertension (PAH). Seventy-nine patients diagnosed with PAH underwent pulmonary DCE imaging at 1.5 T using a time-resolved three-dimensional spoiled gradient echo sequence. The prognostic significance of two DCE parameters, full width at half maximum (FWHM) of the first-pass clearance curve and pulmonary transit time (PTT), along with demographic and invasive catheter measurements, was evaluated by univariate and bivariate Cox proportional hazards regression and Kaplan-Meier analysis. DCE-MR transit times were most closely correlated with cardiac index (CI) and pulmonary vascular resistance index (PVRI) and were both found to be accurate for detecting reduced CI (FWHM area under the curve [AUC] at receiver operating characteristic analysis = 0.91 and PTT AUC = 0.92, respectively) and for detecting elevated PVRI (FWHM AUC = 0.88 and PTT AUC = 0.84, respectively). During the follow-up period, 25 patients died. Patients with longer measurements of FWHM (P = 0.0014) and PTT (P = 0.004) were associated with poor outcome at Kaplan-Meier analysis, and both parameters were strong predictors of adverse outcome from Cox proportional hazards analysis (P = 0.013 and 0.010, respectively). At bivariate analysis, DCE measurements predicted mortality independent of age, gender, and World Health Organization functional class; however, invasive hemodynamic indexes CI, PVRI, and DCE measurements were not independent of one another. In conclusion, DCE-MR transit times predict mortality in patients with PAH and are closely associated with clinical gold standards CI and PVRI.
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Ambrisentan therapy in pulmonary hypertension: clinical use and tolerability in a referral centre.
Ther Adv Respir Dis
PUBLISHED: 04-30-2014
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Ambrisentan is an oral selective endothelin receptor antagonist licensed for use in pulmonary arterial hypertension (PAH). There are few data on clinical use and long-term tolerability in a wider range of patients with pulmonary hypertension (PH).
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Quantitative magnetic resonance imaging of pulmonary hypertension: a practical approach to the current state of the art.
J Thorac Imaging
PUBLISHED: 02-21-2014
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Pulmonary hypertension is a condition of varied etiology, commonly associated with poor clinical outcome. Patients are categorized on the basis of pathophysiological, clinical, radiologic, and therapeutic similarities. Pulmonary arterial hypertension (PAH) is often diagnosed late in its disease course, with outcome dependent on etiology, disease severity, and response to treatment. Recent advances in quantitative magnetic resonance imaging (MRI) allow for better initial characterization and measurement of the morphologic and flow-related changes that accompany the response of the heart-lung axis to prolonged elevation of pulmonary arterial pressure and resistance and provide a reproducible, comprehensive, and noninvasive means of assessing the course of the disease and response to treatment. Typical features of PAH occur primarily as a result of increased pulmonary vascular resistance and the resultant increased right ventricular (RV) afterload. Several MRI-derived diagnostic markers have emerged, such as ventricular mass index, interventricular septal configuration, and average pulmonary artery velocity, with diagnostic accuracy similar to that of Doppler echocardiography. Furthermore, prognostic markers have been identified with independent predictive value for identification of treatment failure. Such markers include large RV end-diastolic volume index, low left ventricular end-diastolic volume index, low RV ejection fraction, and relative area change of the pulmonary trunk. MRI is ideally suited for longitudinal follow-up of patients with PAH because of its noninvasive nature and high reproducibility and is advantageous over other biomarkers in the study of PAH because of its sensitivity to change in morphologic, functional, and flow-related parameters. Further study on the role of MRI image based biomarkers in the clinical environment is warranted.
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Management dilemmas in acute pulmonary embolism.
Thorax
PUBLISHED: 12-16-2013
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Physicians treating acute pulmonary embolism (PE) are faced with difficult management decisions while specific guidance from recent guidelines may be absent.
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Prognostic Value of Cardiovascular Magnetic Resonance Imaging Measurements Corrected for Age and Gender in Idiopathic Pulmonary Arterial Hypertension.
Circ Cardiovasc Imaging
PUBLISHED: 11-25-2013
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-There is limited data on the prognostic value of cardiovascular magnetic resonance (CMR) measurements in idiopathic pulmonary arterial hypertension, with no studies investigating the impact of correction of CMR indices for age and gender on prognostic value.
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emPHasis-10: development of a health-related quality of life measure in pulmonary hypertension.
Eur. Respir. J.
PUBLISHED: 11-14-2013
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To measure the impact of pulmonary hypertension (PH) on health-related quality of life (HRQL), there is a need for a short, validated instrument that can be used in routine clinical practice. Interviews were conducted with 30 patients with PH to derive 32 statements, which were presented as a semantic differential six-point scale (0 - 5), with contrasting adjectives at each end. This item list was completed by patients, attending PH clinics across the UK and Ireland. Rasch analysis was applied to identify items fitting a uni-dimensional model.226 patients (mean age 55.6±14; 70% female) with PH (82% had pulmonary arterial hypertension (PAH) completed the study questionnaires. Ten of the 32 items demonstrated fit to the Rasch model (chi-square =16; p>0.05) and generated the emPHasis-10 questionnaire. Test-retest (ICCC=0.95, n=33) and internal consistency (Chronbachs alpha=0.9) were strong. EmPHasis-10 scores correlated consistently with other relevant measures and discriminated subgroups of patients stratified by WHO functional class (ANOVA F =1.73; p<0.001). The emPHasis-10 is a short, questionnaire for assessing HRQL in PAH. It has excellent measurement properties and sensitive to differences in relevant clinical parameters. It is freely available to use by both the clinical and academic community.
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Oral treprostinil for the treatment of pulmonary arterial hypertension in patients receiving background endothelin receptor antagonist and phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C2 study): a randomized controlled trial.
Chest
PUBLISHED: 05-15-2013
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Treprostinil is a stable prostacyclin analog approved for the treatment of pulmonary arterial hypertension (PAH) as parenteral or inhaled therapy. Treprostinil diolamine, a sustained-release oral formulation of treprostinil, was studied to determine whether it could provide a more convenient prostacyclin treatment option for patients with less severe PAH. The objective of this study was to evaluate the efficacy and safety of oral treprostinil in patients with PAH receiving stable background endothelin receptor antagonist (ERA), phosphodiesterase type 5 inhibitor (PDE-5I) therapy, or both.
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Supplementation of iron in pulmonary hypertension: Rationale and design of a phase II clinical trial in idiopathic pulmonary arterial hypertension.
Pulm Circ
PUBLISHED: 05-11-2013
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Our aim is to assess the safety and potential clinical benefit of intravenous iron (Ferinject) infusion in iron deficient patients with idiopathic pulmonary arterial hypertension (IPAH). Iron deficiency in the absence of anemia (1) is common in patients with IPAH; (2) is associated with inappropriately raised levels of hepcidin, the key regulator of iron homeostasis; and (3) correlates with disease severity and worse clinical outcomes. Oral iron absorption may be impeded by reduced absorption due to elevated hepcidin levels. The safety and benefits of parenteral iron replacement in IPAH are unknown. Supplementation of Iron in Pulmonary Hypertension (SIPHON) is a Phase II, multicenter, double-blind, randomized, placebo-controlled, crossover clinical trial of iron in IPAH. At least 60 patients will be randomized to intravenous ferric carboxymaltose (Ferinject) or saline placebo with a crossover point after 12 weeks of treatment. The primary outcome will be the change in resting pulmonary vascular resistance from baseline at 12 weeks, measured by cardiac catheterization. Secondary measures include resting and exercise hemodynamics and exercise performance from serial bicycle incremental and endurance cardiopulmonary exercise tests. Other secondary measurements include serum iron indices, 6-Minute Walk Distance, WHO functional class, quality of life score, N-terminal pro-brain natriuretic peptide (NT-proBNP), and cardiac anatomy and function from cardiac magnetic resonance. We propose that intravenous iron replacement will improve hemodynamics and clinical outcomes in IPAH. If the data supports a potentially useful therapeutic effect and suggest this drug is safe, the study will be used to power a Phase III study to address efficacy.
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3D contrast-enhanced lung perfusion MRI is an effective screening tool for chronic thromboembolic pulmonary hypertension: results from the ASPIRE Registry.
Thorax
PUBLISHED: 01-24-2013
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Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism potentially curable by surgery. Perfusion scintigraphy is currently advocated as the imaging modality of choice to exclude CTEPH due to its high sensitivity. We have evaluated the diagnostic utility of lung perfusion MRI.
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Noninvasive estimation of PA pressure, flow, and resistance with CMR imaging: derivation and prospective validation study from the ASPIRE registry.
JACC Cardiovasc Imaging
PUBLISHED: 01-17-2013
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The aim of this study was to develop a composite numerical model based on parameters from cardiac magnetic resonance (CMR) imaging for noninvasive estimation of the key hemodynamic measurements made at right heart catheterization (RHC).
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Black blood MRI has diagnostic and prognostic value in the assessment of patients with pulmonary hypertension.
Eur Radiol
PUBLISHED: 07-12-2011
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Double inversion recovery (DIR) "black blood" MRI suppresses the signal from flowing blood, slow flowing blood causes incomplete suppression resulting in pulmonary blood flow artefact (PFA). This study examines the diagnostic utility and prognostic value of a PFA scoring system in a mixed cohort of patients with pulmonary hypertension (PH).
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CT pulmonary angiography combined with echocardiography in suspected systemic sclerosis-associated pulmonary arterial hypertension.
Rheumatology (Oxford)
PUBLISHED: 03-29-2011
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Echocardiography is widely used in the investigation of patients with suspected SSc-associated pulmonary arterial hypertension (SSc-PAH). We investigated whether CT pulmonary angiography (CTPA) provides additive diagnostic value.
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Diagnostic accuracy of contrast-enhanced MR angiography and unenhanced proton MR imaging compared with CT pulmonary angiography in chronic thromboembolic pulmonary hypertension.
Eur Radiol
PUBLISHED: 03-21-2011
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To evaluate the diagnostic accuracy of contrast-enhanced MR angiography (CE-MRA) and the added benefit of unenhanced proton MR angiography compared with CT pulmonary angiography (CTPA) in patients with chronic thromboembolic disease (CTE).
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Paigen diet-fed apolipoprotein E knockout mice develop severe pulmonary hypertension in an interleukin-1-dependent manner.
Am. J. Pathol.
PUBLISHED: 02-23-2011
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Inflammatory mechanisms are proposed to play a significant role in the pathogenesis of pulmonary arterial hypertension (PAH). Previous studies have described PAH in fat-fed apolipoprotein E knockout (ApoE(-/-)) mice. We have reported that signaling in interleukin-1-receptor-knockout (IL-1R1(-/-)) mice leads to a reduction in diet-induced systemic atherosclerosis. We subsequently hypothesized that double-null (ApoE(-/-)/IL-1R1(-/-)) mice would show a reduced PAH phenotype compared with that of ApoE(-/-) mice. Male IL-1R1(-/-), ApoE(-/-), and ApoE(-/-)/IL-1R1(-/-) mice were fed regular chow or a high-fat diet (Paigen diet) for 8 weeks before phenotyping for PAH. No abnormal phenotype was observed in the IL-1R1(-/-) mice. Fat-fed ApoE(-/-) mice developed significantly increased right ventricular systolic pressure and substantial pulmonary vascular remodeling. Surprisingly, ApoE(-/-)/IL-1R1(-/-) mice showed an even more severe PAH phenotype. Further molecular investigation revealed the expression of a putative, alternatively primed IL-1R1 transcript expressed within the lungs but not aorta of ApoE(-/-)/IL-1R1(-/-) mice. Treatment of ApoE(-/-) and ApoE(-/-)/IL-1R1(-/-) mice with IL-1-receptor antagonist prevented progression of the PAH phenotype in both strains. Blocking IL-1 signaling may have beneficial effects in treating PAH, and alternative IL-1-receptor signaling in the lung may be important in driving PAH pathogenesis.
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Computed tomography and magnetic resonance imaging of pulmonary hypertension: Pulmonary vessels and right ventricle.
J Magn Reson Imaging
PUBLISHED: 11-25-2010
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Pulmonary hypertension (PH) is very heterogeneous and the classification identifies five major groups including many associated disease processes. The treatment of PH depends on the underlying cause and accurate classification is paramount. A comprehensive assessment to identify the cause and severity of PH is therefore needed. Furthermore, follow-up assessments are required to monitor changes in disease status and response to therapy. Traditionally, the diagnostic imaging work-up of PH comprised mainly echocardiography, invasive right heart catheterization, and ventilation/perfusion scintigraphy. Due to technical advances, multidetector row computed tomography (CT) and magnetic resonance imaging (MRI) have become important and complementary investigations in the evaluation of patients with suspected PH. Both modalities are reviewed and recommendations for clinical use are given.
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Ventricular mass index correlates with pulmonary artery pressure and predicts survival in suspected systemic sclerosis-associated pulmonary arterial hypertension.
Rheumatology (Oxford)
PUBLISHED: 07-14-2009
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The ventricular mass index (VMI) has been proposed as a diagnostic tool for the assessment of patients with suspected pulmonary hypertension (PH). We hypothesized that in patients with SSc it may predict the presence or absence of PH.
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Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era.
Am. J. Respir. Crit. Care Med.
PUBLISHED: 01-24-2009
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Pulmonary arterial hypertension in association with connective tissue disease (CTD-PAH) has historically had a poor prognosis, with a 1-year survival rate among patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) of 45%. However, more therapies have become available.
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Inhibition of tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) reverses experimental pulmonary hypertension.
J. Exp. Med.
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Pulmonary arterial hypertension (PAH) is a life-threatening disease characterized by the progressive narrowing and occlusion of small pulmonary arteries. Current therapies fail to fully reverse this vascular remodeling. Identifying key pathways in disease pathogenesis is therefore required for the development of new-targeted therapeutics. We have previously reported tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) immunoreactivity within pulmonary vascular lesions from patients with idiopathic PAH and animal models. Because TRAIL can induce both endothelial cell apoptosis and smooth muscle cell proliferation in the systemic circulation, we hypothesized that TRAIL is an important mediator in the pathogenesis of PAH. We demonstrate for the first time that TRAIL is a potent stimulus for pulmonary vascular remodeling in human cells and rodent models. Furthermore, antibody blockade or genetic deletion of TRAIL prevents the development of PAH in three independent rodent models. Finally, anti-TRAIL antibody treatment of rodents with established PAH reverses pulmonary vascular remodeling by reducing proliferation and inducing apoptosis, improves hemodynamic indices, and significantly increases survival. These preclinical investigations are the first to demonstrate the importance of TRAIL in PAH pathogenesis and highlight its potential as a novel therapeutic target to direct future translational therapies.
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Pulmonary hypertension in COPD: results from the ASPIRE registry.
Eur. Respir. J.
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The phenotype and outcome of severe pulmonary hypertension in chronic obstructive pulmonary disease (COPD) is described in small numbers, and predictors of survival are unknown. Data was retrieved for 101 consecutive, treatment-naïve cases of pulmonary hypertension in COPD. Mean ± SD follow-up was 2.3 ± 1.9 years. 59 patients with COPD and severe pulmonary hypertension, defined by catheter mean pulmonary artery pressure ?40 mmHg, had significantly lower carbon monoxide diffusion, less severe airflow obstruction but not significantly different emphysema scores on computed tomography compared to 42 patients with mild-moderate pulmonary hypertension. 1- and 3-year survival for severe pulmonary hypertension, at 70% and 33%, respectively, was inferior to 83% and 55%, respectively, for mild-moderate pulmonary hypertension. Mixed venous oxygen saturation, carbon monoxide diffusion, World Health Organization functional class and age, but not severity of airflow obstruction, were independent predictors of outcome. Compassionate treatment with targeted therapies in 43 patients with severe pulmonary hypertension was not associated with a survival benefit, although improvement in functional class and/or fall in pulmonary vascular resistance >20% following treatment identified patients with improved survival. Standard prognostic markers in COPD have limited value in patients with pulmonary hypertension. This study identifies variables that predict outcome in this phenotype. Despite poor prognosis, our data suggest that further evaluation of targeted therapies is warranted.
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Pulmonary artery relative area change detects mild elevations in pulmonary vascular resistance and predicts adverse outcome in pulmonary hypertension.
Invest Radiol
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The aim of this study was to evaluate the clinical use of magnetic resonance imaging measurements related to pulmonary artery stiffness in the evaluation of pulmonary hypertension (PH).
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Diagnostic accuracy of cardiovascular magnetic resonance imaging of right ventricular morphology and function in the assessment of suspected pulmonary hypertension results from the ASPIRE registry.
J Cardiovasc Magn Reson
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Cardiovascular Magnetic Resonance (CMR) imaging is accurate and reproducible for the assessment of right ventricular (RV) morphology and function. However, the diagnostic accuracy of CMR derived RV measurements for the detection of pulmonary hypertension (PH) in the assessment of patients with suspected PH in the clinic setting is not well described.
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Comparison of the diagnostic utility of cardiac magnetic resonance imaging, computed tomography, and echocardiography in assessment of suspected pulmonary arterial hypertension in patients with connective tissue disease.
J. Rheumatol.
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Pulmonary arterial hypertension (PAH) is a life-threatening complication of connective tissue diseases (CTD). Our aim was to compare the diagnostic utility of noninvasive imaging modalities, i.e., magnetic resonance imaging (MRI), computed tomography (CT), and echocardiography, in evaluation of these patients.
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Serum osteoprotegerin is increased and predicts survival in idiopathic pulmonary arterial hypertension.
Pulm Circ
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We previously reported that osteoprotegerin (OPG) is regulated by pathways associated with pulmonary arterial hypertension (PAH), and is present at elevated levels within pulmonary vascular lesions and sera from patients with idiopathic PAH (IPAH). Since OPG is a naturally secreted protein, we investigated the relationship between serum OPG and disease severity and outcome in patients with IPAH and animal models. OPG mRNA expression was measured in pulmonary artery smooth muscle cells (PASMC) from pulmonary arteries of patients with and without IPAH. Serum concentrations of OPG were measured in a retrospective and prospective group of patients. OPG levels were compared with phenotypic data and other putative PAH biomarkers. Prognostic significance was assessed and levels compared with healthy controls. Correlation of OPG and pulmonary vascular remodeling was also performed in rodent models of PAH. OPG mRNA was significantly increased 2-fold in PASMC isolated from explanted PAH lungs compared with control. Serum OPG concentrations were markedly elevated in IPAH compared with controls. In Cohort 1 OPG levels significantly correlated with mean right atrial pressure and cardiac index, while in Cohort 2 significant correlations existed between age-adjusted OPG levels and gas transfer. In both cohorts an OPG concentration above a ROC-derived threshold of 4728 pg/ml predicted poorer survival. In two rodent models, OPG correlated with the degree of pulmonary vascular remodeling. OPG levels are significantly elevated in patients with idiopathic PAH and are of prognostic significance. The role of OPG as a potential biomarker and therapeutic target merits further investigation.
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Lung morphology assessment with balanced steady-state free precession MR imaging compared with CT.
Radiology
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To evaluate the utility of 1.5-T noncontrast magnetic resonance (MR) imaging of the lung parenchyma and to compare it with computed tomography (CT) in the assessment of interstitial lung disease and other morphologic lung abnormalities.
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JoVE Visualize is a tool created to match the last 5 years of PubMed publications to methods in JoVE's video library.

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In developing our video relationships, we compare around 5 million PubMed articles to our library of over 4,500 methods videos. In some cases the language used in the PubMed abstracts makes matching that content to a JoVE video difficult. In other cases, there happens not to be any content in our video library that is relevant to the topic of a given abstract. In these cases, our algorithms are trying their best to display videos with relevant content, which can sometimes result in matched videos with only a slight relation.