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Pulmonary hypertension treatment

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https://www.readbyqxmd.com/read/28636457/notch-signaling-in-lung-diseases
#1
Jiehan Jiang, Kui Xiao, Ping Chen
NOTCH is a fundamental signaling system that regulates normal embryonic development and tissue homeostasis in adult life. NOTCH receptor is a single-pass transmembrane protein normally triggered via direct cell-to-cell contact, in which NOTCH ligands bind the extracellular domain of the receptor, inducing γ-secretase cleavage and release of intracellular domain. The intracellular domain binds to the transcriptional effector RBPJκ to activate transcription of target genes that regulate cell differentiation, patterning, and morphogenesis during embryonic development and adult life...
June 21, 2017: Experimental Lung Research
https://www.readbyqxmd.com/read/28634521/a-review-of-echocardiograms-in-hypertensive-patients-greater-than-60-years-in-a-community-based-family-medicine-program
#2
Shideh Doroudi, Michael D DeLisi, Vincent A DeBari
Background: Heart disease as a result of Hypertension is known to occur. Anatomical and functional changes of the heart can easily be detected by echocardiography, which is a safe and readily available study. Objectives: The aims of this study were to evaluate the prevalence of common echocardiographic changes in chronic hypertensive patients and to compare these changes in male and female populations. Design/methods: The study was a community-based cross-sectional study, on 227 hypertensive patients, 60 years and older, seen in St...
January 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28634363/long-term-health-outcomes-in-patients-with-prader-willi-syndrome-a-nationwide-cohort-study-in-denmark
#3
E Hedgeman, S P Ulrichsen, S Carter, N C Kreher, K P Malobisky, M M Braun, J Fryzek, M S Olsen
BACKGROUND: Prader-Willi syndrome (PWS) is a rare congenital disease that affects growth, sexual development, cognitive function and behavior. Individuals exhibit food preoccupation and hyperphagia, which may lead to obesity with premature morbidity and mortality. The aim of this work was to evaluate the risk of venous thromboembolisms (VTE), myocardial infarction, pulmonary hypertension, sleep apnea, depression, anxiety and all-cause mortality among persons with PWS as compared to an age- and sex-matched general population cohort...
June 21, 2017: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/28633267/perventricular-implantation-of-melody-valve-in-child-with-pulmonary-hypertension-after-a-potts-shunt
#4
Tomasz Mroczek, Marcin Demkow, Tomasz Moszura, Aleksandra Morka, Janusz Skalski
The Potts operation is a surgical option for the treatment of children with suprasystemic pulmonary arterial hypertension (PAH). Its positive hemodynamic effect may be obliterated by pulmonary valvar insufficiency. We describe a case in which the Potts shunt was followed by perventricular Melody valve implantation in the pulmonary position in a 12-kg child with a previously repaired truncus arteriosus communis (TAC) in whom PAH developed. The combined approach using both procedures significantly improved functional capacity and led to spectacular reverse remodeling of the right ventricle seen at 2-year follow-up...
July 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28629920/pulmonary-hypertension-in-parenchymal-lung-diseases-any-future-for-new-therapies
#5
REVIEW
Sergio Harari, Davide Elia, Marc Humbert
Pulmonary hypertension (PH) due to chronic lung diseases is associated with a poor prognosis, regardless of the underlying respiratory condition. Updated PH guidelines recommend optimal treatment of the underlying lung disease, including long-term oxygen therapy, in patients with chronic hypoxaemia despite the lack of randomized controlled clinical trials supporting this statement. So far, randomized controlled trials on drugs approved for pulmonary arterial hypertension (PAH) have yielded discouraging results in both interstitial lung diseases (ILD) and chronic obstructive pulmonary diseases (COPD) with PH...
June 16, 2017: Chest
https://www.readbyqxmd.com/read/28628863/simultaneous-quantification-of-endothelin-receptor-antagonists-and-phosphodiesterase-5-inhibitors-currently-used-in-pulmonary-arterial-hypertension
#6
Yeliz Enderle, Lukas Witt, Heinrike Wilkens, Ekkehard Grünig, Walter E Haefeli, Jürgen Burhenne
Combination treatment with endothelin receptor antagonists (ERA) and phosphodiesterase 5 inhibitors (PDE5I) improved efficacy of pulmonary arterial hypertension (PAH) therapy. However, drug-drug interactions, variable exposure, non-adherence can influence plasma levels. For these reasons, drug quantification may be advantageous particularly in patients with poor treatment responses. We developed, validated, and applied an assay for the simultaneous quantification of ambrisentan, bosentan, macitentan, sildenafil, and tadalafil as well as their main (and partly active) metabolites in human plasma...
June 10, 2017: Journal of Pharmaceutical and Biomedical Analysis
https://www.readbyqxmd.com/read/28628347/preventive-treatment-with-atorvastatin-ameliorates-endothelial-dysfunction-of-small-pulmonary-arteries-in-monocrotaline-induced-pulmonary-hypertensive-rats
#7
Zhihong Lin, Zhi Jiang, Xiaodong Huang, Xiaoqi Cai, Huajun Wang, Liangdi Xie
This study examined the effects of preventive atorvastatin (Ator) treatment on vasodilatation of small pulmonary arteries (SPAs) in monocrotaline (MCT)-induced pulmonary hypertensive rats. SD rats were randomly assigned to: normal control (Ctr), pulmonary arterial hypertension (PAH), PAH treated with 5 mg/kg/d Ator (LAtor), or 10 mg/kg/d Ator (HAtor). PAH was induced by MCT injection (40 mg/kg, i.p.). Mean pulmonary artery pressure (mPAP), right ventricular hypertrophy index (RVHI%), endothelium-dependent relaxations (EDdRs), and endothelium-independent relaxations (EDiRs) were determined...
June 19, 2017: Clinical and Experimental Hypertension: CHE
https://www.readbyqxmd.com/read/28628189/-a-statement-the-polish-cardiac-society-working-group-on-pulmonary-circulation-on-screening-for-cteph-patients-after-acute-pulmonary-embolism
#8
Michał Ciurzyński, Marcin Kurzyna, Grzegorz Kopeć, Piotr Błaszczak, Łukasz Chrzanowski, Karol Kamiński, Katarzyna Mizia-Stec, Tatiana Mularek-Kubzdela, Ewa Mroczek, Andrzej Biederman, Piotr Pruszczyk, Adam Torbicki
Both pharmacological and invasive treatment of chronic thromboembolic pulmonary hypertension (CTEPH) is now available in Poland and the awareness of the disease among physicians is growing. Thus, the Polish Cardiac Society's Working Group on Pulmonary Circulation in cooperation with independent experts in this field, have launched the statement on algorithm to guide a CTEPH diagnosis in patients with previous acute pulmonary embolism (APE). In Poland, every year this disease affects about 250 patients. CTEPH should be suspected in individuals after APE with dyspnea, despite at least 3 months period of effective anticoagulation, particularly when specified risk factors are present...
2017: Kardiologia Polska
https://www.readbyqxmd.com/read/28627661/altered-expression-of-ppar%C3%A2-%C3%AE-and-trpc-in-neonatal-rats-with-persistent-pulmonary-hypertension
#9
Yanna Du, Jianhua Fu, Li Yao, Lin Qiao, Na Liu, Yujiao Xing, Xindong Xue
Persistent pulmonary hypertension of the newborn (PPHN) is a life‑threatening disease that is commonly observed in the neonatal intensive care unit. PPHN is pathologically characterized by pulmonary vascular remodeling and, in particular, pulmonary artery smooth muscle cell (PASMC) proliferation. Decreased expression levels of peroxisome proliferator‑activated receptor γ (PPAR‑γ), which is a member of the nuclear receptor hormone superfamily, in combination with elevated expressions of transient receptor potential cation channel, subfamily C, member 1 (TRPC1) and TRPC6 contributes to the PASMC proliferation and excessive pulmonary vascular remodeling in adult pulmonary hypertension (PH)...
June 9, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28626631/chronic-eosinophilic-pneumonia-adjunctive-therapy-with-inhaled-steroids
#10
Christopher Chan, David DeLapp, Perry Nystrom
Idiopathic chronic eosinophilic pneumonia (ICEP) is a rare form of diffuse parenchymal lung disease first identified by Carrington et al. in 1969. It is characterized by the presence of constitutional and respiratory symptoms with associated peripheral opacities on imaging and elevated serum and/or bronchoalveolar eosinophilia. Although data is limited regarding etiology or prevalence, it is known that ICEP has a 2:1 female: male predominance and typically affects non-smokers. Diagnosis rests on the clinical constellation of respiratory symptoms of at least 2-4 weeks duration, the presence of diffuse pulmonary alveolar consolidation, classically described as the "photographic negative of pulmonary edema", the presence of eosinophils ≥40% on bronchoalveolar lavage or ≥1000/mm(3) eosinophils on peripheral blood and the exclusion of other known causes of eosinophilic lung diseases such as drugs, toxins, fungi, parasites, and collagen-vascular disorders...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28626480/development-of-dry-powder-inhaler-containing-tadalafil-loaded-plga-nanoparticles
#11
Jaleh Varshosaz, Somayeh Taymouri, Hamed Hamishehkar, Razieh Vatankhah, Shadi Yaghubi
Inhalable dry powders containing poly lactic-co-glycolic acid (PLGA) nanoparticles (NPs) were developed for the delivery of tadalafil (TAD) for treatment of life-treating pulmonary arterial hypertension. Taguchi design was employed to evaluate the effects of different formulation variables on the physicochemical characteristics of PLGA-NPs prepared using emulsion solvent evaporation method. Inhalable PLGA-NPs of TAD were successfully prepared by co-spray drying the PLGA-NPs with inert carriers. Physicochemical characteristics and in vitro deposition of the aerosolized drug were also evaluated...
June 2017: Research in Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28624000/aneurysms-of-the-azygos-vein
#12
REVIEW
Maximilian Kreibich, Matthias Siepe, Jochen Grohmann, Gregor Pache, Friedhelm Beyersdorf
OBJECTIVE: Azygos vein aneurysms (AVAs) are uncommon and infrequently diagnosed. When confronted with a patient presenting with an AVA, physicians can rely on only a few case reports after an extensive literature search. To date, no guideline, no rule, and no review on the optimal treatment strategy for these patients exist. METHODS: A PubMed and MEDLINE database search for papers and case reports describing AVA was performed. Cases from our own institutions were also reviewed...
July 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28621173/evaluation-and-management-approaches-for-scleroderma-lung-disease
#13
Sara R Schoenfeld, Flavia V Castelino
Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are leading causes of morbidity and mortality in systemic sclerosis (SSc). As symptoms are often under-reported in SSc, early screening of ILD and PAH is of paramount importance, and early treatment may be associated with better clinical outcomes. Serologies are particularly helpful in identifying patients at risk for pulmonary involvement. Pulmonary function testing, high-resolution computed tomography of the chest and echocardiography are important tools in the initial screening of these patients...
June 1, 2017: Therapeutic Advances in Respiratory Disease
https://www.readbyqxmd.com/read/28619957/chronic-pulmonary-aspergillosis-complicating-sarcoidosis
#14
Yurdagül Uzunhan, Hilario Nunes, Florence Jeny, Maxime Lacroix, Sophie Brun, Pierre-Yves Brillet, Emmanuel Martinod, Marie-France Carette, Diane Bouvry, Caroline Charlier, Fanny Lanternier, Carole Planès, Abdellatif Tazi, Olivier Lortholary, Robert P Baughman, Dominique Valeyre
Chronic pulmonary aspergillosis (CPA) complicating sarcoidosis (SA) is associated with high mortality, and there is a lack of clarity regarding the relative contributions of SA or CPA.This was a retrospective single-centre study on CPA-SA.In total, 65 patients (44 men), aged 41.4±13.5 and 48.3±11.9 years at the time of SA and CPA diagnoses, respectively, were included between 1980 and 2015. Of these, 64 had fibrocystic SA, most often advanced, with composite physiological index (CPI) >40 (65% of patients) and pulmonary hypertension (PH) (31%), and 41 patients (63%) were treated for SA (corticosteroids or immunosuppressive drugs)...
June 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28619123/chd-and-respiratory-syncytial-virus-global-expert-exchange-recommendations
#15
Robert M R Tulloh, Constancio Medrano-Lopez, Paul A Checchia, Claudia Stapper, Naokata Sumitomo, Matthias Gorenflo, Eun Jung Bae, Antonio Juanico, Juan M Gil-Jaurena, Mei-Hwan Wu, Talal Farha, Ali Dodge-Khatami, Rocky Tsang, Gerard Notario, Colleen Wegzyn
BACKGROUND: Palivizumab is the standard immunoprophylaxis against serious disease due to respiratory syncytial virus infection. Current evidence-based prophylaxis guidelines may not address certain children with CHD within specific high-risk groups or clinical/management settings. METHODS: An international steering committee of clinicians with expertise in paediatric heart disease identified key questions concerning palivizumab administration; in collaboration with an additional international expert faculty, evidence-based recommendations were formulated using a quasi-Delphi consensus methodology...
June 16, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28616649/earlier-pulmonary-valve-replacement-in-down-syndrome-patients-following-tetralogy-of-fallot-repair
#16
Rachel T Sullivan, Peter C Frommelt, Garick D Hill
The association between Down syndrome and pulmonary hypertension could contribute to more severe pulmonary regurgitation after tetralogy of Fallot repair and possibly earlier pulmonary valve replacement. We compared cardiac magnetic resonance measures of pulmonary regurgitation and right ventricular dilation as well as timing of pulmonary valve replacement between those with and without Down syndrome after tetralogy of Fallot repair. Review of our surgical database from 2000 to 2015 identified patients with tetralogy of Fallot with pulmonary stenosis...
June 14, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28616089/severe-pulmonary-arterial-hypertension-associated-with-congenital-cardiac-shunts-evolution-under-specific-treatment
#17
R I Negoi, I Ghiorghiu, F Filipoiu, M Hostiuc, I Negoi, C Ginghina
Objective: The aim of this study was to compare the effects of Sildenafil, Bosentan and combined therapy in patients with congenital cardiac shunts associated pulmonary artery hypertension (CCS-PAH). Design: Prospective observational study (February 2011 - January 2014) with a historical control group (January 2009 - January 2011). Setting: "CC Iliescu" Institute for Emergency Cardiovascular Diseases of Bucharest, a tertiary university-affiliated center. Patients: All cases with CCS-PAH. Interventions: Specific vasodilatory therapy: Sildenafil, Bosentan or combined therapy...
April 2017: Journal of Medicine and Life
https://www.readbyqxmd.com/read/28610669/riociguat-for-pulmonary-arterial-hypertension-and-chronic-thromboembolic-pulmonary-hypertension-results-from-a-phase-ii-long-term-extension-study
#18
Michael Halank, Marius M Hoeper, Hossein-Ardeschir Ghofrani, F Joachim Meyer, Gerd Stähler, Jürgen Behr, Ralf Ewert, Monique Fletcher, Pablo Colorado, Sylvia Nikkho, Friedrich Grimminger
BACKGROUND: Riociguat was well tolerated and improved exercise and functional capacity in patients with pulmonary arterial hypertension (PAH) and inoperable chronic thromboembolic pulmonary hypertension (CTEPH) in a 12-week Phase II trial. We present final data from the long-term extension phase of this study. METHODS: During this multicenter, open-label, uncontrolled long-term extension study, riociguat dose could be changed at the physician's discretion (range 0...
July 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28606655/mortality-in-pulmonary-arterial-hypertension-due-to-congenital-heart-disease-serial-changes-improve-prognostication
#19
M T U Schuijt, I M Blok, A H Zwinderman, A C M J van Riel, M J Schuuring, R J de Winter, A L Duijnhouwer, A P J van Dijk, B J M Mulder, B J Bouma
BACKGROUND: Adult patients with pulmonary arterial hypertension due to congenital heart disease (PAH-CHD) suffer from high mortality. This underlines the importance of adequate risk stratification to guide treatment decisions. Several baseline parameters are associated with mortality, however, their prognostic value may weaken after years of follow-up. Therefore we investigated the prognostic value of serial changes in standard clinical parameters in PAH-CHD. METHODS: In this prospective observational cohort study we included consecutive PAH-CHD adults, between 2005 and 2016...
June 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28606232/-a-clinical-analysis-of-15-children-with-systemic-lupus-erythematosus-accompanied-by-pulmonary-hypertension
#20
Ji Li, Jing-Ran Ma, Zhi-Xing Sun, Jing-Jing Jiang, Yan-Qing Dong, Qian Wang, Hong-Mei Song
OBJECTIVE: To evaluate the clinical features, laboratory findings, diagnosis and treatment, and prognosis of children with systemic lupus erythematosus (SLE) accompanied by pulmonary hypertension (PH). METHODS: The clinical symptoms, laboratory findings, echocardiographic features, SLE disease activity index, and treatment outcome of 15 hospitalized children with SLE accompanied by PH were retrospectively analyzed. RESULTS: Among the 15 patients, the median interval from diagnosis of SLE to diagnosis of PH was 0...
June 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
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