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https://www.readbyqxmd.com/read/28735030/effect-of-bosentan-therapy-in-persistent-pulmonary-hypertension-of-the-newborn
#1
Gunlawadee Maneenil, Anucha Thatrimontrichai, Waricha Janjindamai, Supaporn Dissaneevate
BACKGROUND: Persistent pulmonary hypertension of the newborn (PPHN) contributes to neonatal hypoxemia and is associated with a high mortality. Some PPHN patients are unresponsive to inhaled nitric oxide (iNO). Bosentan, an oral endothelin-1 receptor antagonist, reduces pulmonary vascular resistance and hence may play a role in the treatment of PPHN. METHODS: A retrospective medical records review was performed in newborns who received oral bosentan as an adjunctive therapy for treatment of PPHN between January 2013 and February 2016 at the neonatal intensive care unit of Songklanagarind Hospital...
July 11, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28734831/effects-of-all-oral-anti-viral-therapy-on-hvpg-and-systemic-hemodynamics-in-patients-with-hepatitis-c-virus-associated-cirrhosis
#2
Sabela Lens, Edilmar Alvarado, Zoe Mariño, María-Carlota Londoño, Elba LLop, Javier Martinez, Jose Ignacio Fortea, Luís Ibañez, Xavier Ariza, Anna Baiges, Adolfo Gallego, Rafael Bañares, Angela Puente, Agustín Albillos, Jose Luís Calleja, Xavier Torras, Virginia Hernández-Gea, Jaume Bosch, Cándid Villanueva, Xavier Forns, Juan Carlos García-Pagán
BACKGROUND & AIMS: Patients with hepatitis C virus (HCV)-associated cirrhosis and clinical significant portal hypertension (CSPH, hepatic venous pressure gradient [HVPG] 10 mmHg or greater), despite achieving sustained virological response (SVR) to therapy, remain at risk of liver decompensation. We investigated hemodynamic changes following SVR in patients with CSPH and whether liver stiffness measurements (LSMs) can rule out the presence of CSPH. METHODS: We performed a multicenter prospective study of 226 patients with HCV-associated cirrhosis and CSPH who had SVR to interferon-free therapy at 6 Liver Units in Spain...
July 19, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28734808/-congenital-cytomegalovirus-infection-manifesting-as-neonatal-respiratory-distress-in-an-hiv-exposed-uninfected-newborn
#3
A Pham, H El Mjati, N Nathan, F Kieffer, D Mitanchez
BACKGROUND: Cytomegalovirus (CMV) is one of the most common intrauterine infections, affecting approximately 1% of all live births. There are few reports on congenital CMV infections manifesting as isolated pneumonitis. CASE REPORT: We report a case of congenital CMV with neonatal respiratory distress affecting an HIV-exposed uninfected infant. This infant required noninvasive ventilation beginning within the first 15min of life. The initial chest X-ray showed diffuse bilateral ground-glass opacifications...
July 19, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28725332/thyrotoxic-valvulopathy-case-report-and-review-of-the-literature
#4
Keniel Pierre, Sushee Gadde, Bassam Omar, G Mustafa Awan, Christopher Malozzi
We report a 42-year-old female who was admitted for abdominal pain, and also endorsed dyspnea, fatigue and chronic palpitations. Past medical history included asthma, patent ductus arteriosus repaired in childhood and ill-defined thyroid disease. Physical examination revealed blood pressure of 136/88 mm Hg and heart rate of 149 beats per minute. Cardiovascular exam revealed an irregularly irregular rhythm, and pulmonary exam revealed mild expiratory wheezing. Abdomen was tender. Electrocardiogram revealed atrial fibrillation with rapid ventricular response which responded to intravenous diltiazem...
June 2017: Cardiology Research
https://www.readbyqxmd.com/read/28702718/single-center-experience-using-selexipag-in-a-pediatric-population
#5
Roberto Gallotti, Diana E Drogalis-Kim, Gary Satou, Juan Alejos
Pulmonary arterial hypertension (PAH) is a rare and progressive disorder. Current treatment in the pediatric population includes phosphodiesterase 5 inhibitors (PDE-5i), endothelin receptor antagonists (ERA), and both inhaled and intravenous prostacyclin pathway agonists. As of December 22, 2015 the first oral prostacyclin pathway agonist, selexipag (Uptravi(®)), was FDA approved in the US. In this case series, we discuss our single-center experience using selexipag in a pediatric population, composed of both patients with idiopathic PAH, and patients with congenital heart disease and PAH...
July 13, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28686445/discovery-and-optimization-of-chromeno-2-3-c-pyrrol-9-2h-ones-as-novel-selective-and-orally-bioavailable-phosphodiesterase-5-inhibitors-for-the-treatment-of-pulmonary-arterial-hypertension
#6
Deyan Wu, Tianhua Zhang, Yiping Chen, Yadan Huang, Haiju Geng, Yanfa Yu, Chen Zhang, Zengwei Lai, Yinuo Wu, Xiaolei Guo, Jianwen Chen, Hai-Bin Luo
Phosphodiesterase 5 (PDE5) inhibitors have been used as clinical agents to treat erectile dysfunction and pulmonary arterial hypertension (PAH). Herein, we detail the discovery of a novel series of chromeno[2,3-c]pyrrol-9(2H)-one derivatives as selective and orally bioavailable inhibitors against phosphodiesterase 5. Medicinal chemistry optimization resulted in 2, which exhibits a desirable inhibitory potency of 5.6 nM with remarkable selectivity as well as excellent pharmacokinetic properties and an oral bioavailability of 63...
July 7, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28680576/recommendations-for-the-use-of-oral-treprostinil-in-clinical-practice-a-delphi-consensus-project-pulmonary-circulation
#7
Franck F Rahaghi, Jeremy P Feldman, Roblee P Allen, Victor Tapson, Zeenat Safdar, Vijay P Balasubramanian, Shelley Shapiro, Michael A Mathier, Jean M Elwing, Murali M Chakinala, R James White
Oral treprostinil was recently labeled for treatment of pulmonary arterial hypertension. Similar to the period immediately after parenteral treprostinil was approved, there is a significant knowledge gap for practicing physicians who might prescribe oral treprostinil. Despite its oral route of delivery, use of the drug is challenging because of the requirement for careful titration and management of drug-related adverse effects. We aimed to create a consensus document combining available evidence with expert opinion to provide guidance for use of oral treprostinil...
March 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28676038/efficacy-and-safety-of-oral-sildenafil-in-children-with-down-syndrome-and-pulmonary-hypertension
#8
Maurice Beghetti, Andrzej Rudzinski, Min Zhang
BACKGROUND: Despite the increased risk for pulmonary hypertension in children with Down syndrome, the response to treatment with targeted therapies for pulmonary hypertension in these patients is not well characterized. The Sildenafil in Treatment-naive children, Aged 1-17 years, with pulmonary arterial hypertension (STARTS-1) trial was a dose-ranging study of the short-term efficacy and safety of oral sildenafil in children with pulmonary arterial hypertension. We assessed the safety and efficacy of oral sildenafil in children with Down syndrome and pulmonary arterial hypertension...
July 4, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28639216/biocomparison-study-of-adult-and-paediatric-dose-strengths-of-the-prostacyclin-receptor-agonist-selexipag
#9
Margaux Boehler, Shirin Bruderer, Ivan Ulč, Jasper Dingemanse
BACKGROUND AND OBJECTIVES: Selexipag is an oral, non-prostanoid, selective prostacyclin receptor agonist recently marketed for the treatment of pulmonary arterial hypertension (PAH) in adults. Selexipag may also be an effective treatment in children with PAH. The aim of this study was to compare the pharmacokinetics of selexipag and its active metabolite ACT-333679 following single oral administration of one tablet of 200 µg selexipag (Treatment A) vs. 4 paediatric tablets of 50 µg (Treatment B) in healthy adult male subjects...
June 21, 2017: European Journal of Drug Metabolism and Pharmacokinetics
https://www.readbyqxmd.com/read/28624000/aneurysms-of-the-azygos-vein
#10
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/28597780/novel-approach-to-classifying-patients-with-pulmonary-arterial-hypertension-using-cluster-analysis
#11
Kishan S Parikh, Youlan Rao, Tariq Ahmad, Kai Shen, G Michael Felker, Sudarshan Rajagopal
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 clinical variables of PAH patients from the FREEDOM-M, FREEDOM-C, and FREEDOM-C2 randomized trials of oral treprostinil versus placebo. Participants were either treatment-naïve (FREEDOM-M) or on background therapy (FREEDOM-C, FREEDOM-C2)...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597752/withdrawal-of-long-term-epoprostenol-therapy-in-pulmonary-arterial-hypertension-pah
#12
George Calcaianu, Mihaela Calcaianu, Matthieu Canuet, Irina Enache, Romain Kessler
Once initiated for pulmonary arterial hypertension (PAH), epoprostenol treatment usually needs to be delivered for an indefinite duration. It is possible that some participants could be transitioned from epoprostenol to oral therapies. We retrospectively evaluated eight PAH participants transitioned from epoprostenol to PAH oral drugs. The criteria for epoprostenol withdrawal were: (1) persistent improvement of clinic and hemodynamic status; (2) stable dose of epoprostenol for the last three months; and (3) the participant's preference for oral therapy after evaluation of risk-benefit...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28587325/sildenafil-suppresses-the-proliferation-and-enhances-the-apoptosis-of-hemangioma-endothelial-cells
#13
Xiaopo He, Yiran Liu, Kai Li, Aijun Yang, Rui Wang, Shaohua Liu
Treatment of infantile hemangiomas (IH) with propranolol was first reported in 2008. Regressions of lymphatic malformations combined with pulmonary hypertension was first reported in 2012 after three children received treatment with oral sildenafil, which serves as an antagonist of phosphodiesterase isoform-5 (PDE-5). A marked expression of endothelial cells in the cytoplasm of IH tissues was obtained in our previous study. Therefore, the present study hypothesized that the antagonist of PDE-5, sildenafil, may lead to the regression of hemangiomas...
June 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28568509/investigation-and-treatment-for-iron-deficiency-in-heart-failure-the-unmet-need-in-lower-and-middle-income-countries
#14
REVIEW
Abel Makubi, David J Roberts
Frank iron deficiency has been associated with a wide range of cardiac and pulmonary abnormalities including non-ischaemic cardiomyopathy. Iron deficiency anaemia and isolated iron deficiency are well-defined adverse prognostic factors in non-ischaemic cardiac failure. Furthermore, iron-deficient patients in chronic heart failure with a serum ferritin of <100 μg/l or <300 μg/l with reduced transferrin saturation of <20%, who were given intravenous iron showed improved clinical outcomes. Iron deficiency with or without anaemia affects over a quarter of the world's population, but the impact of iron deficiency in heart failure and the effective management of iron deficiency in heart failure in Lower and Middle Income Countries (LMICs) is not well described...
June 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28556581/population-modeling-of-selexipag-pharmacokinetics-and-clinical-response-parameters-in-patients-with-pulmonary-arterial-hypertension
#15
A Krause, M Machacek, D Lott, N Hurst, S Bruderer, J Dingemanse
Selexipag (Uptravi) is an oral selective IP prostacyclin receptor agonist approved for the treatment of pulmonary arterial hypertension (PAH). The pivotal GRIPHON study was the largest clinical study ever conducted in PAH patients, providing long-term data from 1,156 patients. PAH comedication did not affect exposure to selexipag, while exposure to its active metabolite ACT-333679 was reduced by 30% when taken in combination, clinically not relevant in the context of individual dose up-titration. Using log-linear regression models linking model-predicted steady-state exposure to pharmacodynamics (PD), exposure to selexipag and ACT-333679 showed some statistically significant, albeit not clinically relevant, effects on exercise capacity, laboratory values, and the occurrence of prostacyclin-related adverse events, but not on vital signs or adverse events denoting hemorrhage...
May 27, 2017: CPT: Pharmacometrics & Systems Pharmacology
https://www.readbyqxmd.com/read/28533253/selexipag-for-the-treatment-of-pulmonary-arterial-hypertension
#16
Zachary R Noel, Kazuhiko Kido, Tracy E Macaulay
PURPOSE: The pharmacology, pharmacokinetics, clinical efficacy, safety and tolerability, dosing and administration, and place in therapy of selexipag, an orally administered selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension (PAH), are reviewed. SUMMARY: The first-in-class oral prostacyclin IP receptor agonist selexipag (Uptravi, Actelion Pharmaceuticals) was approved by the Food and Drug Administration in December 2015...
May 22, 2017: American Journal of Health-system Pharmacy: AJHP
https://www.readbyqxmd.com/read/28524738/selexipag-a-selective-prostacyclin-receptor-agonist-in-pulmonary-arterial-hypertension-a-pharmacology-review
#17
REVIEW
Jesús Honorato Pérez
Pulmonary hypertension is defined by a mean pulmonary artery pressure ≥25 mm Hg at rest. Management of pulmonary arterial hypertension (PAH) includes specific drug therapy with calcium channel blockers in vasoreactive patients, or drugs approved for PAH in non-reactive patients that target the endothelin, nitric-oxide and prostacyclin pathways. Areas covered: The review covers receptor selectivity, pharmacokinetics, pharmacodynamics and adverse effects (AEs) of intravenous (IV) epoprostenol (synthetic prostacyclin); the prostacyclin analogs iloprost, beraprost, and treprostinil administered by IV, subcutaneous, inhaled or oral routes; and the oral selective prostacyclin receptor agonist selexipag...
July 2017: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/28523018/effect-of-azithromycin-in-combination-with-simvastatin-in-the-treatment-of-chronic-obstructive-pulmonary-disease-complicated-by-pulmonary-arterial-hypertension
#18
Peidong Wang, Jie Yang, Yanwei Yang, Zhixin Ding
OBJECTIVE: To evaluate the effect of azithromycin in combination with simvastatin in the treatment of chronic obstructive pulmonary disease (COPD) complicated by pulmonary arterial hypertension. METHODS: Eighty-six patients who developed COPD and pulmonary arterial hypertension and received treatment from August 2013 to October 2014 were selected and randomly divided into an observation group and a control group using random number table, 43 in each group. Patients in the control group were orally administrated 20 mg of simvastatin, once a day...
March 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28522783/splenic-abcesses-as-infectious-complication-following-127-implantation-of-left-ventricular-asssist-device-case-report
#19
Sławomir Gajda, Anna M Szczepanik, Grzegorz Religa, Andrzej Misiak, Andrzej B Szczepanik
Left ventricular assist device (LVAD) is one of the modern management therapies in patients with advanced heart failure, and it serves as a bridge to heart transplantation or even as destination therapy. However, it is burdened with a high risk of thromboembolic, hemorrhagic, and infectious complications despite prophylactic management. Splenic abscesses, as septic complications following implantation of mechanical ventricular support, have not yet been described in the literature. We report of a patient with severe left ventricular insufficiency (NYHA II/III), pulmonary hypertension, and arrhythmia who underwent implantation of the Heart Ware® pump for left ventricular support with simultaneous tricuspidvalvoplasty, as a bridge therapy to heart transplantation...
February 28, 2017: Polski Przeglad Chirurgiczny
https://www.readbyqxmd.com/read/28494686/clinical-pharmacology-efficacy-and-safety-of-selexipag-for-the-treatment-of-pulmonary-arterial-hypertension
#20
REVIEW
Shirin Bruderer, Noémie Hurst, Tatiana Remenova, Jasper Dingemanse
Selexipag is the first oral, non-prostanoid, selective prostacyclin receptor (IP receptor) agonist, approved for the long-term treatment of pulmonary arterial hypertension (PAH) in adult patients. Areas covered: This article reviews the clinical pharmacology, efficacy, and safety of selexipag in the treatment of PAH. Expert opinion: Selexipag is the first oral drug that selectively targets the prostacyclin pathway, and has evidence of long-term efficacy and safety. In the global phase 3 study GRIPHON (NCT01106014) in PAH patients, selexipag significantly reduced the risk of the primary composite outcome of morbidity/mortality (M/M)...
June 2017: Expert Opinion on Drug Safety
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