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Pulmonary arterial hypertension

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https://www.readbyqxmd.com/read/29222289/what-is-the-role-of-screening-for-pulmonary-hypertension-in-adults-and-children-with-sickle-cell-disease
#1
REVIEW
Shaina M Willen, Mark T Gladwin
Patient case: An 18-year-old male patient with homozygous hemoglobin SS disease was evaluated for progressive dyspnea and elevated tricuspid regurgitant jet velocity (TRV) on echocardiography. The patient's case is described in detail in Lancet1 He had been treated with regular transfusions since childhood for stroke, had rare episodes of vaso-occlusive pain episodes, and did not take narcotic pain medications. He presented with progressive severe dyspnea on exertion and lower extremity edema. His laboratory tests were notable for a total hemoglobin level of 11...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222288/cardiovascular-complications-in-patients-with-sickle-cell-disease
#2
REVIEW
Mark T Gladwin
Sickle cell disease (SCD) is an autosomal recessive disease in which homozygosity for a single point mutation in the gene encoding the β-globin chain produces hemoglobin S molecules that polymerize within the erythrocyte during deoxygenation; the result is sustained hemolytic anemia and vaso-occlusive events. As patients live to adulthood, the chronic impact of sustained hemolytic anemia and episodic vaso-occlusive episodes leads to progressive end-organ complications. This scenario culminates in the development of 1 or more major cardiovascular complications of SCD for which there are no approved or consensus therapies...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29221952/danshensu-prevents-hypoxic-pulmonary-hypertension-in-rats-by-inhibiting-the-proliferation-of-pulmonary-artery-smooth-muscle-cells-via-tgf-%C3%AE-smad3-associated-pathway
#3
Ning Zhang, Mingqing Dong, Ying Luo, Feng Zhao, Yongjun Li
Hypoxic pulmonary hypertension is characterized by the remodeling of pulmonary artery. Previously we showed that tanshinone IIA, one lipid-soluble component from the Chinese herb Danshen, ameliorated hypoxic pulmonary hypertension by inhibiting pulmonary artery remodeling. Here we explored the effects of danshensu, one water-soluble component of Danshen, on hypoxic pulmonary hypertension and its mechanism. Rats were exposed to hypobaric hypoxia for 4 weeks to develop hypoxic pulmonary hypertension along with administration of danshensu...
December 5, 2017: European Journal of Pharmacology
https://www.readbyqxmd.com/read/29221480/prevalence-and-etiologies-of-pulmonary-hypertension-in-africa-a-systematic-review-and-meta-analysis
#4
Jean Joel Bigna, Jean Jacques Noubiap, Jobert Richie Nansseu, Leopold Ndemnge Aminde
BACKGROUND: Despite the recent increasing worldwide attention towards pulmonary hypertension (PH), its epidemiology remains poorly described in Africa. Accordingly, we performed a systematic review and meta-analysis of PH prevalence, incidence and etiologies in Africa. METHODS: We searched PubMed, EMBASE, African Journals Online, and Africa Index Medicus. Published observational studies until September 20, 2017, including adult participants residing in Africa were considered...
December 8, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29221285/robot-assisted-thoracic-surgery-for-complex-procedures
#5
Shuenn-Wen Kuo, Pei-Ming Huang, Mong-Wei Lin, Ke-Cheng Chen, Jang-Ming Lee
Background: As an option for minimally invasive thoracic surgery, robot-assisted thoracic surgery (RATS) has shown comparable perioperative outcomes to those achieved by traditional video-assisted thoracic surgery (VATS). It is unknown whether RATS might have any potential benefits in more complex thoracic surgical procedures, which usually require open surgery instead of VATS. The current study presents a preliminary result regarding the use of RATS in complex thoracic operations in an attempt to address this unresolved question...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29218116/erratum-new-insights-and-new-hope-for-pulmonary-arterial-hypertension-natriuretic-peptides-clearance-receptor-as-a-novel-therapeutic-target-for-a-complex-disease
#6
Emmanuel Eroume-A Egom, Tiam Feridooni, Rebabonye B Pharithi, Barkat Khan, Haaris A Shiwani, Vincent Maher, Yassine El Hiani, Robert A Rose, Kishore Bs Pasumarthi, Hilaire A Ribama
[This corrects the article on p. 112 in vol. 9, PMID: 28951773.].
2017: International Journal of Physiology, Pathophysiology and Pharmacology
https://www.readbyqxmd.com/read/29217526/first-in-human-phase-i-study-of-single-agent-vanucizumab-a-first-in-class-bi-specific-anti-ang-2-anti-vegf-antibody-in-adult-patients-with-advanced-solid-tumors
#7
Manuel Hidalgo, Maria Martinez-Garcia, Christophe Le Tourneau, Christophe Massard, Elena Garralda, Valentina Boni, Alvaro Taus, Joan Albanell, Marie-Paule Sablin, Marie Alt, Rastilav Bahleda, Andreea Varga, Christophe Boetsch, Izolda Franjkovic, Florian Heil, Angelika Lahr, Katharina Lechner, Anthony Morel, Tapan K Nayak, Simona Rossomanno, Kevin Smart, Kay-Gunnar Stubenrauch, Oliver Krieter
PURPOSE: Vanucizumab is an investigational anti-angiogenic, first-in-class, bi-specific monoclonal antibody targeting VEGF-A and angiopoietin-2 (Ang-2). This first-in-human study evaluated the safety, pharmacokinetics, pharmacodynamics, and antitumor activity of vanucizumab in adults with advanced solid tumors refractory to standard therapies. EXPERIMENTAL DESIGN: Patients received escalating bi-weekly (q2w; 3-30 mg/kg) or weekly (qw; 10-30 mg/kg) intravenous doses guided by a Bayesian logistic regression model with overdose control...
December 7, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/29216632/perturbations-in-endothelial-dysfunction-associated-pathways-in-the-nitrofen-induced-congenital-diaphragmatic-hernia-model
#8
Siqin Zhaorigetu, Henry Bair, Jonathan Lu, Di Jin, Scott D Olson, Matthew T Harting
Although it is well known that nitrofen induces congenital diaphragmatic hernia (CDH), including CDH-associated lung hypoplasia and pulmonary hypertension (PH) in rodents, the mechanism of pathogenesis remains largely unclear. It has been reported that pulmonary artery (PA) endothelial cell (EC) dysfunction contributes to the development of PH in CDH. Thus, we hypothesized that there is significant alteration of endothelial dysfunction-associated proteins in nitrofen-induced CDH PAs. Pregnant SD rats received either nitrofen or olive oil on gestational day 9...
December 8, 2017: Journal of Vascular Research
https://www.readbyqxmd.com/read/29215546/initial-tadalafil-and-ambrisentan-combination-therapy-in-pulmonary-arterial-hypertension-clinical-and-haemodynamic-long-term-efficacy-italy-study
#9
Michele D'Alto, Emanuele Romeo, Paola Argiento, Giuseppe Paciocco, Renato Prediletto, Stefano Ghio, Michele Correale, Francesco Lo Giudice, Roberto Badagliacca, Alessandra Greco, Carmine Dario Vizza
AIMS: Initial combination therapy with ambrisentan and tadalafil (upfront therapy) offers clinical benefits in pulmonary arterial hypertension (PAH) and reduces the risk of clinical failure compared with monotherapy in naïve patients. The aim of study is to assess the efficacy of a 12-month upfront therapy with ambrisentan and tadalafil in improving haemodynamics in incident PAH patients. METHODS: This is a multicentre retrospective analysis of real-world Italian clinical data in 56 patients with newly diagnosed PAH...
January 2018: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/29214548/first-clinical-symptom-as-a-prognostic-factor-in-systemic-sclerosis-results-of-a-retrospective-nationwide-cohort-study
#10
Manuel Rubio-Rivas, Xavier Corbella, Melany Pestaña-Fernández, Carles Tolosa-Vilella, Alfredo Guillen-Del Castillo, Dolores Colunga-Argüelles, Luis Trapiella-Martínez, Nerea Iniesta-Arandia, María Jesús Castillo-Palma, Luis Sáez-Comet, María Victoria Egurbide-Arberas, Norberto Ortego-Centeno, Mayka Freire, Jose Antonio Vargas-Hitos, Juan José Ríos-Blanco, Jose Antonio Todolí-Parra, Mónica Rodríguez-Carballeira, Adela Marín-Ballvé, Pablo Segovia-Alonso, Xavier Pla-Salas, Ana Belén Madroñero-Vuelta, Manuel Ruiz-Muñoz, Vicent Fonollosa-Pla, Carmen Pilar Simeón-Aznar, E Callejas Moraga, E Calvo, C Carbonell, M J Castillo, A J Chamorro, D Colunga, X Corbella, M V Egurbide, G Espinosa, V Fonollosa, M Freire, F J García Hernández, R González León, A Guillén Del Castillo, N Iniesta, R Lorenzo, A B Madroñero, B Marí, A Marín, N Ortego-Centeno, M Pérez Conesa, M Pestaña, X Pla, J J Ríos Blanco, M Rodríguez Carballeira, M Rubio Rivas, M Ruiz Muñoz, L Sáez Comet, P Segovia, C P Simeón, A Soto, E Tarí, J A Todolí, C Tolosa, L Trapiella, J A Vargas Hitos, G Verdejo
The objective of the study is to determine the importance of the mode of onset as prognostic factor in systemic sclerosis (SSc). Data were collected from the Spanish Scleroderma Registry (RESCLE), a nationwide retrospective multicenter database created in 2006. As first symptom, we included Raynaud's phenomenon (RP), cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and digestive hypomotility. A total of 1625 patients were recruited...
December 7, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29212971/-chronic-myeloid-leukemia-complicated-by-pulmonary-hypertension-during-dasatinib-therapy-a-single-center-retrospective-study
#11
Yoko Edahiro, Tomoiku Takaku, Hakuoh Konishi, Yutaka Tsukune, Isao Fujioka, Kiyoshi Takasu, Akihiko Gotoh, Hiroyuki Daida, Norio Komatsu
Pulmonary artery hypertension (PAH) has been reported to be a severe adverse event associated with dasatinib therapy. Among the 76 chronic myeloid patients who were treated with dasatinib at our hospital, six patients showed high estimated pulmonary arterial systolic pressure, as observed by echocardiography. PAH was confirmed using right heart catheterization in three (3.9%) patients with increased mean pulmonary artery pressure (mPAP). In one patient, although mPAP was higher than the normal range, it did not fulfill the criteria of pulmonary hypertension...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29212876/egr-1-mediates-leptin-induced-ppar%C3%AE-reduction-and-proliferation-of-pulmonary-artery-smooth-muscle-cells
#12
Xinming Xie, Shaojun Li, Yanting Zhu, Lu Liu, Rui Ke, Jian Wang, Xin Yan, Lan Yang, Li Gao, Weijin Zang, Manxiang Li
Loss of peroxisome proliferator-activated receptor γ (PPARγ) has been found to contribute to pulmonary artery smooth muscle cell (PASMC) proliferation and pulmonary arterial remodeling therefore the development of pulmonary hypertension (PH). Yet, the molecular mechanisms underlying PPARγ reduction in PASMC remain poorly understood. Here, we demonstrated that leptin dose- and time-dependently inducued PPARγ down-regulation and proliferation of primary cultured rat PASMC, this was accompanied with the activation of extracellular regulated kinase1/2 (ERK1/2) signaling pathway and subsequent induction of early growth response-1 (Egr-1) expression...
December 6, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/29212831/impaired-pulmonary-arterial-vasoconstriction-and-nitric-oxide-mediated-relaxation-underlie-severe-pulmonary-hypertension-in-sugen-hypoxia-rat-model
#13
Helen Christou, Hannes Hudalla, Zoe Michael, Evgenia Filatava, Jun Li, Minglin Zhu, Jose Possomato-Vieira, Carlos Dias-Junior, Stella Kourembanas, Raouf A Khalil
Pulmonary vasoreactivity could determine the responsiveness to vasodilators and in turn the prognosis of pulmonary hypertension (PH). We hypothesized that pulmonary vasoreactivity is impaired, and examined the underlying mechanisms, in the sugen-hypoxia rat model of severe PH. Male Sprague-Dawley rats were injected with sugen (20 mg/kg sc) and exposed to hypoxia (9% O2) for 3 weeks followed by 4 weeks in normoxia (Su/Hx), or treated with sugen alone (Su) or hypoxia alone (Hx) or neither (Nx). After hemodynamic measurements, the heart was assessed for right ventricular hypertrophy (Fulton's Index), the pulmonary artery, aorta and mesenteric arteries were isolated for vascular function studies, and contractile markers were measured in pulmonary artery using quantitative PCR...
December 6, 2017: Journal of Pharmacology and Experimental Therapeutics
https://www.readbyqxmd.com/read/29212800/extracellular-retention-of-pdgf-b-directs-vascular-remodeling-in-mouse-hypoxia-induced-pulmonary-hypertension
#14
Philip Tannenberg, Ya-Ting Chang, Lars Muhl, Bàrbara Laviña, Hanna Gladh, Guillem Genové, Christer Betsholtz, Erika Folestad, Karin Tran-Lundmark
Pulmonary hypertension (PH) is a lethal condition and current vasodilator therapy has limited effect. Anti-proliferative strategies targeting platelet-derived growth factor (PDGF) receptors, such as imatinib, have generated promising results in animal studies. Imatinib is however a non-specific tyrosine kinase inhibitor and has in clinical studies caused unacceptable adverse events. Further studies are needed on the role of PDGF signaling in PH. Here, mice expressing a variant of PDGF-B with no retention motif (Pdgfbret/ret), resulting in defective binding to extracellular matrix, were studied...
December 6, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29210771/effect-of-inhaled-nitric-oxide-on-hemodynamics-in-lambs-with-1%C3%A2-ventricle-circulation
#15
Hitoshi Kanamitsu, Yasuhiro Fujii, Luca Centola, Katsushi Kinouchi, Liqun Zhu, Robert K Riemer, Olaf Reinhartz
Inhaled nitric oxide (NO) is widely used to treat postoperative pulmonary hypertension in congenital heart disease. It is believed that NO increases cardiac output (CO) by decreasing pulmonary vascular resistance (PVR), leading to increased left ventricular preload. However, the effect of NO on CO in patients with 1½ ventricle circulation remains unclear. To evaluate this, a superior cavopulmonary (SCP) shunt was constructed in 10 juvenile sheep. A PTFE graft was inserted between the superior vena cava (SVC) and the main pulmonary artery (PA)...
November 27, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/29208691/inhibition-of-nitric-oxide-synthase-unmasks-vigorous-vasoconstriction-in-established-pulmonary-arterial-hypertension
#16
Mariko Tanaka, Kohtaro Abe, Masahiko Oka, Keita Saku, Keimei Yoshida, Tomohito Ishikawa, Ivan F McMurtry, Kenji Sunagawa, Sumio Hoka, Hiroyuki Tsutsui
It is widely accepted that impaired bioavailability of endothelial nitric oxide (NO) plays a critical role in the pathophysiology of pulmonary arterial hypertension (PAH). However, there are published data that show that relatively many PAH patients respond favorably to acetylcholine-induced pulmonary vasodilation during their follow-up period, when diverse stages of the disorder are included. We hypothesized that NO bioavailability varies depending on the progression of PAH Adult rats were exposed to the VEGF receptor blocker Sugen5416 and 3 weeks of hypoxia followed by return to normoxia for various additional weeks...
December 2017: Physiological Reports
https://www.readbyqxmd.com/read/29206084/identity-crisis-in-pulmonary-arterial-hypertension
#17
G Ruffenach, S Bonnet, S Rousseaux, S Khochbin, S Provencher, F Perros
Pulmonary arterial hypertension (PAH) shares many hallmarks with cancer. Cancer cells acquire their hallmarks by a pathological Darwinian evolution process built on the so-called cancer cell "identity crisis." Here we demonstrate that PAH shares the most striking features of the cancer identity crisis: the ectopic expression of normally silent tissue-specific genes.
January 2018: Pulmonary Circulation
https://www.readbyqxmd.com/read/29203759/-right-hemicolectomy-under-spinal-anesthesia-due-to-cancer-of-ascending-large-bowel-case-report
#18
Gniewomir Ćwiertnia, Michał Dyaczyński
Surgery procedures of the abdomen cavity are commonly performed in general anaesthesia. Patients from high risk group with circulatory insufficiency, respiratory failure pose a problem. They undergo surgical procedures for life indications and emergency cases. Regional anaesthesia can be an alternative for general anaesthesia, and makes planned surgical treatment possible for this group of patients. The study presents the case of 79-year-old male with chronic obstructive pulmonary disease, after left lung upper lobectomy, arterial hypertension, who underwent operation due to ascending large bowel cancer under spinal anaesthesia as planned...
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29203267/the-significance-of-diminished-stweak-and-p-selectin-content-in-platelets-of-patients-with-pulmonary-arterial-hypertension
#19
Remigiusz Kazimierczyk, Piotr Błaszczak, Krzysztof Kowal, Małgorzata Jasiewicz, Małgorzata Knapp, Anna Szpakowicz, Katarzyna Ptaszyńska-Kopczyńska, Bożena Sobkowicz, Ewa Waszkiewicz, Ryszard Grzywna, Włodzimierz J Musial, Karol A Kamiński
Pulmonary arterial hypertension (PAH) is a progressive disease characterized by proliferative changes in pulmonary arteries. There is growing evidence suggesting that soluble tumor necrosis factor-like weak inducer of apoptosis (sTWEAK) and P-selectin could be involved in PAH development and progression. Here we investigate whether circulating platelets may be a source of sTWEAK and contribute to diminished availability of sTWEAK and P-selectin in PAH patients. We have prospectively enrolled two independent study groups of stable patients with confirmed PAH and age matched controls: derivation (10 PAH; 15 controls) and validation (20 PAH; 12 controls)...
December 1, 2017: Cytokine
https://www.readbyqxmd.com/read/29202826/autonomic-nervous-system-involvement-in-pulmonary-arterial-hypertension
#20
REVIEW
Mylène Vaillancourt, Pamela Chia, Shervin Sarji, Jason Nguyen, Nir Hoftman, Gregoire Ruffenach, Mansoureh Eghbali, Aman Mahajan, Soban Umar
Pulmonary arterial hypertension (PAH) is a chronic pulmonary vascular disease characterized by increased pulmonary vascular resistance (PVR) leading to right ventricular (RV) failure. Autonomic nervous system involvement in the pathogenesis of PAH has been demonstrated several years ago, however the extent of this involvement is not fully understood. PAH is associated with increased sympathetic nervous system (SNS) activation, decreased heart rate variability, and presence of cardiac arrhythmias. There is also evidence for increased renin-angiotensin-aldosterone system (RAAS) activation in PAH patients associated with clinical worsening...
December 4, 2017: Respiratory Research
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