keyword
https://read.qxmd.com/read/38494833/effect-of-off-label-targeted-drugs-on-long-term-survival-in-chronic-thromboembolic-pulmonary-hypertension-insights-from-a-national-multicentre-prospective-registry
#1
JOURNAL ARTICLE
Wanying Xia, Yuling Qian, Yangyi Lin, Ruilin Quan, Yuanhua Yang, Zhenwen Yang, Hongyan Tian, Shengqing Li, Jieyan Shen, Yingqun Ji, Qing Gu, Huijun Han, Changming Xiong, Jianguo He
BACKGROUND AND OBJECTIVE: Off-label pulmonary arterial hypertension (PAH)-targeted drugs are commonly prescribed for non-operated chronic thromboembolic pulmonary hypertension (CTEPH), but their effect on the long-term prognosis of CTEPH remains unknown. This study investigated the effect of off-label PAH-targeted drugs on the long-term survival of CTEPH patients. METHODS: CTEPH patients were enrolled from a prospective multicentre national registry. Except for licensed riociguat and treprostinil, other PAH-targeted drugs were off-label...
March 17, 2024: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://read.qxmd.com/read/38488130/comparative-adherence-of-macitentan-versus-ambrisentan-and-bosentan-in-australian-patients-with-pulmonary-arterial-hypertension-a-retrospective-real-world-database-study
#2
JOURNAL ARTICLE
Edmund Lau, Eugene Kotlyar, Yogeshwar Makanji, Dae Young Yu, Jin Yu Tan, Jeremy Casorso, Mahsa H Kouhkamari, Sooyeol Lim, David Bin-Chia Wu, Paul Bloomfield
Aim: Bosentan, ambrisentan, and macitentan are endothelin receptor antagonists (ERAs), currently available in Australia for treatment of pulmonary arterial hypertension (PAH). This study assessed the comparative adherence of these ERAs for PAH in Australian patients. Methods: This retrospective, observational study used data for adults with PAH from the Services Australia 10% Pharmaceuticals Benefits Scheme (PBS) dataset (01/2006-10/2020). The primary outcome was treatment adherence (ie, receiving ≥80% of ERA doses over 12 months)...
March 15, 2024: Journal of Medical Economics
https://read.qxmd.com/read/38485279/-cyp7a1-gene-induction-via-shp-dependent-or-independent-mechanisms-can-increase-the-risk-of-drug-induced-liver-injury-independently-or-in-synergy-with-bsep-inhibition
#3
JOURNAL ARTICLE
Congrong Niu, Xiaodong Xie, Renmeng Liu, Xiaomin Liang, Yiding Hu, Yurong Lai
Drug-induced liver injury (DILI) is a frequent cause of clinical trial failures during drug development. While inhibiting BSEP is a well-documented DILI mechanism, interference with genes related to bile acid (BA) metabolism and transport can further complicate DILI development. Here, the effects of twenty-eight compounds on genes associated with BA metabolism and transport were evaluated, including those with discontinued development or use, boxed warnings, and clean labels for DILI. The study also included rifampicin and omeprazole, PXR and AhR ligands, and four mitogen-activated protein kinase kinase (MEK1/2) inhibitors...
March 14, 2024: Drug Metabolism and Disposition: the Biological Fate of Chemicals
https://read.qxmd.com/read/38464344/actigraphy-methodology-in-the-kids-mod-pah-trial-physical-activity-as-a-functional-endpoint-in-pediatric-clinical-trials
#4
JOURNAL ARTICLE
Catherine M Avitabile, Usha S Krishnan, Delphine Yung, Stephanie S Handler, Nidhy Varghese, Angela Bates, Jeff Fineman, Rachel Sullivan, Grace Friere, Eric Austin, Mary P Mullen, Carol Pereira, Eric J Christensen, Gayane Yenokyan, Joseph M Collaco, Steven H Abman, Lew Romer, D Dunbar Ivy, Erika B Rosenzweig
Pulmonary vasodilator treatment can improve hemodynamics, right ventricular function, symptoms, and survival in pediatric pulmonary hypertension (PH). However, clinical trial data are lacking due to many constraints. One major limitation is the lack of relevant trial endpoints reflective of hemodynamics or functional status in patients in whom standard exercise testing is impractical, unreliable, or not reproducible. The Kids Mod PAH trial (Mono- vs. Duo Therapy for Pediatric Pulmonary Arterial Hypertension) is an ongoing multicenter, Phase III, randomized, open-label, pragmatic trial to compare the safety and efficacy of first-line combination therapy (sildenafil and bosentan) to first-line monotherapy (sildenafil alone) in 100 pediatric patients with PH across North America...
January 2024: Pulmonary Circulation
https://read.qxmd.com/read/38399397/impact-of-a-moderate-cyp3a4-inducer-bosentan-on-lurbinectedin-pharmacokinetics-and-safety-in-patients-with-advanced-solid-tumors-an-open-label-two-way-crossover-phase-ib-drug-drug-interaction-study
#5
JOURNAL ARTICLE
Irene Moreno, Tatiana Hernández, Emiliano Calvo, Salvador Fudio, Carmen Kahatt, Cristian Fernández, Jorge Luis Iglesias, Gema Corral, Laura Pérez-Ramos, Lola Montilla, Ali Zeaiter, Rubin Lubomirov
This open-label, two-way, crossover, phase Ib drug-drug interaction study investigated whether the pharmacokinetics (PKs) and safety profile of lurbinectedin (LRB) are affected by co-administration of a moderate CYP3A4 inducer (bosentan, BOS) in adult patients with advanced solid tumors. Eleven patients were randomly assigned to Sequence 1 (LRB + BOS in Cycle 1 [C1] and LRB alone in Cycle 2 [C2]) or Sequence 2 (LRB alone in C1 and LRB + BOS in C2), and finally, eight patients (four per sequence) were considered evaluable for PK assessment...
January 30, 2024: Pharmaceuticals
https://read.qxmd.com/read/38379558/case-report-extracorporeal-life-support-as-a-successful-bridge-to-recovery-in-an-incident-case-of-pulmonary-arterial-hypertension
#6
Benjamin Pequignot, Ari Chaouat, François Chabot, Bruno Levy, Simon Valentin
Pulmonary arterial hypertension (PAH) is characterized by a progressive increase in pulmonary vascular resistance (PVR) due to vascular remodeling of the small pulmonary arteries. In advanced RV failure or severe hypoxemia, extra corporeal life support (ECLS) is now to be considered, with the objective to bridge patients back to their baseline clinical state while waiting or right after lung transplantation, or bridge to pharmacological optimization of PAH (i.e., bridge to recovery). We describe herein a case of a 30-year-old woman ( gravida 6, para 6) with an incident case of heritable PAH revealed by refractory hypoxemia...
2024: Frontiers in Medicine
https://read.qxmd.com/read/38378970/treatment-of-pulmonary-arterial-hypertension-in-patients-with-connective-tissue-diseases-a-systematic-review-and-meta-analysis
#7
JOURNAL ARTICLE
Mustafa Erdogan, Sinem Nihal Esatoglu, Burcak Kilickiran Avci, Gulen Hatemi
The evidence for the treatment of connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) mostly depends on subgroup or post hoc analysis of randomized controlled trials (RCTs). Thus, we performed a meta-analysis of RCTs that reported outcomes for CTD-PAH. PubMed and EMBASE were searched for CTD-PAH treatment. The selected outcomes were functional class (FC) change, survival rates, 6-min walk distance (6-MWD), clinical worsening (CW), N-terminal prohormone BNP (NT-proBNP), pulmonary vascular resistance (PVR), mean pulmonary arterial pressure (mPAP), right atrial pressure (RAP), and cardiac index (CI)...
February 20, 2024: Internal and Emergency Medicine
https://read.qxmd.com/read/38375723/bosentan-and-ambrisentan-in-the-treatment-of-idiopathic-pulmonary-fibrosis-a-meta-analysis
#8
JOURNAL ARTICLE
H-F Li, J-X Wang, Z-F Xie, L-H Li, B Li, F-F Huang, J Li, X-L Zhou
OBJECTIVE: The aim is to showcase the effectiveness and safety of bosentan or ambrisentan in individuals diagnosed with idiopathic pulmonary fibrosis (IPF) and offer fresh evidence for the management of this condition. MATERIALS AND METHODS: For this research, we conducted a meta-analysis of randomized controlled trials by searching various databases, including the Cochrane Library, Excerpta Medica Database, PubMed, and Web of Science. The retrieval was conducted until November 2021...
February 2024: European Review for Medical and Pharmacological Sciences
https://read.qxmd.com/read/38375585/how-do-risk-evaluation-and-mitigation-strategies-impact-clinical-practice-a-national-survey-of-physicians
#9
JOURNAL ARTICLE
Ameet Sarpatwari, Zhigang Lu, Massimiliano Russo, Heidi Zakoul, Su Been Lee, Gita A Toyserkani, Esther H Zhou, Cynthia LaCivita, Katherine Hyatt Hawkins Shaw, Laura Zendel, Gerald J Dal Pan, Aaron S Kesselheim
The US Food and Drug Administration can require risk evaluation and mitigation strategy (REMS) programs for prescription drugs to ensure the benefits of use outweigh the risks. We conducted a national survey of physicians' experiences prescribing eight REMS-covered drugs: (1) ambrisentan; (2) bosentan; (3) clozapine; (4) isotretinoin; (5-7) the multiple myeloma (MM) drugs lenalidomide, pomalidomide, thalidomide; and (8) sodium oxybate. Between May 2022 and January 2023, we surveyed 5,331 physician prescribers of these drugs, and 1,295 (24%) returned surveys (range: 149 for bosentan to 226 for MM drugs)...
February 20, 2024: Clinical Pharmacology and Therapeutics
https://read.qxmd.com/read/38368315/clinical-course-of-copd-patients-with-exercise-induced-elevation-of-pulmonary-artery-pressure-or-less-severe-pulmonary-hypertension-presenting-with-respiratory-symptoms-and-the-impact-of-bosentan-intervention-prospective-single-center-randomized-parallel-group
#10
RANDOMIZED CONTROLLED TRIAL
Takeru Kashiwada, Yosuke Tanaka, Toru Tanaka, Tetsuya Okano, Yoshinobu Saito, Masahiro Seike, Mitsunori Hino, Hiroshi Kimura, Akihiko Gemma
BACKGROUND: The data on bosentan were lacking for the treatment of exercise-induced elevation of pulmonary artery pressure (eePAP) or less severe PH in COPD. This study was conducted to investigate long-term efficacy and safety of bosentan for the treatment of eePAP or less severe PH in COPD. METHODS: COPD patients diagnosed at this hospital as having COPD (WHO functional class II, III or IV) with eePAP or less severe PH whose respiratory symptoms were stable but remained and gradually progressed even after COPD therapy were randomly assigned in a 1:1 ratio to receive either bosentan or no PH treatment for two years and assessed at baseline and every 6 months for respiratory failure, activities of daily living (ADL), lung and heart functions by right heart catheterization (RHC), and other parameters...
February 17, 2024: BMC Pulmonary Medicine
https://read.qxmd.com/read/38343873/venoarterial-extracorporeal-membrane-oxygenation-for-vasoplegic-shock-after-treprostinil-refill-of-an-implanted-intravenous-pump-a-case-report
#11
Lucía Valencia, Sergio López, Ana Olivas, Ángel Becerra, María Desirée Alemán-Segura, Marta Évora-García, Nazario Ojeda, Leonardo Cabrera, Aurelio Rodríguez-Pérez, Gregorio Pérez-Peñate
INTRODUCTION: Venoarterial extracorporeal membrane oxygenation (ECMO) is a rescue therapy that can stabilize patients with hemodynamic compromise. Indications continue to evolve, including drug overdose. However, the indication merely for vasoplegic shock following drug overdose is controversial. CASE SUMMARY: We report a case of a 57-year-old male with high-risk idiopathic pulmonary arterial hypertension treated with upfront triple combination therapy (sildenafil, bosentan, and intravenous treprostinil infusion via subcutaneous abdominal implantable pump)...
2024: Frontiers in Cardiovascular Medicine
https://read.qxmd.com/read/38329072/bosentan-in-the-treatment-of-persistent-pulmonary-hypertension-in-newborns-a-systematic-review-and-meta-analysis
#12
JOURNAL ARTICLE
Ning Gao, Yuanyuan Lv, Yanbin Cui, Pengchun Wang, Xin He
BACKGROUND: Persistent pulmonary hypertension of the newborn is a life-threatening condition that affects about 1-2 per 1,000 live births worldwide. Bosentan is an oral dual endothelin receptor antagonist that may have a beneficial effect on persistent pulmonary hypertension of the newborn by reducing pulmonary vascular resistance and improving oxygenation. However, its role in persistent pulmonary hypertension of the newborn remains unclear. OBJECTIVES: To systematically evaluate the efficacy and safety of bosentan as an adjuvant therapy for persistent pulmonary hypertension of the newborn in newborns...
February 8, 2024: Cardiology in the Young
https://read.qxmd.com/read/38306796/systematic-literature-review-to-inform-the-portuguese-recommendations-for-the-management-of-raynaud-s-phenomenon-and-digital-ulcers-in-systemic-sclerosis-and-other-connective-tissue-diseases
#13
JOURNAL ARTICLE
Emanuel Costa, Filipe Cunha-Santos, Eduardo Dourado, Daniela Oliveira, Louise Falzon, Vasco Romão, Ana Catarina Duarte, Ana Cordeiro, Tânia Santiago, Alexandre Sepriano
OBJECTIVE: To perform a systematic literature review (SLR) aimed at evaluating the efficacy and safety of pharmacological and non-pharmacological treatments for Raynaud's phenomenon (RP) and digital ulcers (DU) in patients with systemic sclerosis (SSc) and other connective tissue diseases (CTD), in order to inform the Portuguese recommendations for managing RP and DU in these patients. METHODS: A SLR was conducted until May 2022 to identify studies assessing the efficacy and safety of pharmacological and non-pharmacological interventions for RP and DU in SSc and other CTD...
February 2, 2024: ARP Rheumatol
https://read.qxmd.com/read/38276220/current-management-and-future-directions-for-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#14
REVIEW
Ahmed K Mahmoud, Mohammed Tiseer Abbas, Moaz A Kamel, Juan M Farina, Milagros Pereyra, Isabel G Scalia, Timothy Barry, Chieh-Ju Chao, Francois Marcotte, Chadi Ayoub, Robert L Scott, David S Majdalany, Reza Arsanjani
Current management of patients with congenital heart disease has increased their survival into adulthood. This is accompanied by potential cardiac complications, including pulmonary hypertension associated with congenital heart disease (PAH-CHD). PAH-CHD constitutes a challenging subgroup of pulmonary hypertension and requires expert management to improve quality of life and prognosis. Novel agents have shown a significant improvement in morbidity and mortality in patients with pulmonary arterial hypertension...
December 20, 2023: Journal of Personalized Medicine
https://read.qxmd.com/read/38241693/effect-of-benidipine-alone-and-in-combination-with-bosentan-and-sildenafil-in-amelioration-of-pulmonary-arterial-hypertension-in-experimental-model-in-rats
#15
JOURNAL ARTICLE
Kalpana Kumari, Vishal Kumar Vishwakarma, Kuldeep Kumar, Asit Ranjan Mridha, Sudhir Kumar Arava, Sameer Dhingra, Nirmal Singh, Harlokesh Narayan Yadav
Pulmonary arterial hypertension (PAH) is a persistent condition affecting the pulmonary arteries' endothelium. Benidipine, a calcium channel blocker, possesses vasodilatory, anti-inflammatory activity, reduces oxidative stress, and inhibits the activity of TGF-β receptor 1 and α-SMA. The present study was designed to investigate the effect of benidipine alone and in combination with bosentan and sildenafil on monocrotaline (MCT)-induced pulmonary hypertension (PH) in a rat model. PAH was induced by single-dose administration of MCT in rats...
January 17, 2024: Journal of Cardiovascular Pharmacology
https://read.qxmd.com/read/38179676/liver-injury-due-to-endothelin-receptor-antagonists-a-real-world-study-based-on-post-marketing-drug-monitoring-data
#16
JOURNAL ARTICLE
Shichao Dong, Xiaofei Guo, Huayu Wang, Chuan Sun
BACKGROUND: Liver injury is the hallmark adverse reaction of endothelin receptor antagonist (ERA). Since the first drug, bosentan has been widely used in clinical practice, hepatotoxicity has been accompanied by the history of ERA. The new ERA has been proven to have a lower liver risk but the current research findings are inconsistent. ERA-based targeted drug combinations are commonly used in the treatment of pulmonary arterial hypertension, where the risk of liver injury is difficult to estimate...
2024: Therapeutic Advances in Respiratory Disease
https://read.qxmd.com/read/38145828/role-of-endothelin-et-a-receptors-in-the-hypertension-induced-by-the-vegfr-2-kinase-inhibitors-axitinib-and-lenvatinib-in-conscious-freely-moving-rats
#17
JOURNAL ARTICLE
Patrizia Pannucci, Marieke Van Daele, Samantha L Cooper, Edward S Wragg, Julie March, Marleen Groenen, Stephen J Hill, Jeanette Woolard
Receptor tyrosine kinase inhibitors (RTKIs) suppress tumour growth by targeting vascular endothelial growth factor receptor 2 (VEGFR-2) which is an important mediator of angiogenesis. Here, we demonstrate that two potent RTKIs, axitinib and lenvatinib, are associated with hypertensive side effects. Doppler flowmetry was used to evaluate regional haemodynamic profiles of axitinib and lenvatinib. Male Sprague Dawley rats (350-500 g) were instrumented with Doppler flow probes (renal and mesenteric arteries and descending abdominal aorta) and catheters (jugular vein and distal abdominal aorta, via the caudal artery)...
December 23, 2023: Biochemical Pharmacology
https://read.qxmd.com/read/37991630/comparative-treatment-persistence-and-adherence-to-endothelin-receptor-antagonists-among-patients-with-pulmonary-arterial-hypertension-in-japan-a-real-world-administrative-claims-database-study
#18
JOURNAL ARTICLE
Junichi Omura, Yogeshwar Makanji, Nobuhiro Tanabe, Dae Young Yu, Jin Yu Tan, Sooyeol Lim, Mahsa H Kouhkamari, Jeremy Casorso, David Bin-Chia Wu, Paul Bloomfield
INTRODUCTION: Real-world data on the comparative effectiveness of endothelin receptor antagonists (ERAs; macitentan, bosentan, ambrisentan) for pulmonary arterial hypertension (PAH), particularly in Asian countries, are scarce. We evaluated the persistence of these ERAs before and after macitentan approval in Japan (2015). METHODS: We used real-world data from the Japanese Medical Data Vision administrative claims database between April 2008 and November 2020. Patients with PAH were identified from the dataset...
November 22, 2023: Pulmonary Therapy
https://read.qxmd.com/read/37981276/screening-and-evaluation-of-the-hit-compound-from-a-dna-encoded-library-derived-from-natural-products-based-on-immobilized-endothelin-receptor-a
#19
JOURNAL ARTICLE
Yajun Zhang, Li Lin, Sai Qiao, Xue Zhao, Ting Li, Qi Liang
The enormous growing demand for drug candidates binding to endothelin receptor A (ETA ) has made it necessary to continuously pursue new strategies for ligand screening and early evaluation. This work achieved the one-step immobilization of ETA based on the bioorthogonal chemistry between the epidermal growth factor receptor tag (EGFR-tag) and ibrutinib. Comprehensive characterizations including Western blot analysis are performed to realize the morphology, antibody/ligand recognition activity, and specificity of the immobilized ETA ...
November 17, 2023: International Journal of Biological Macromolecules
https://read.qxmd.com/read/37980887/endothelin-receptor-blockade-improves-cerebral-blood-flow-mediated-dilation-in-a-mouse-model-of-alzheimer-s-disease
#20
JOURNAL ARTICLE
Daniel Henrion, Philippe Bonnin, Emilie Vessieres, Anne-Laure Guihlot, Marc Iglarz, Bernard I Lévy
INTRODUCTION: Cerebral blood flow (CBF) is reduced in patients with Alzheimer's disease (AD). Flow-mediated dilation (FMD), which plays a key role in the regulation of blood flow, is attenuated by endothelin-1. We hypothesized that endothelin receptor blockade may improve CBF in AD. METHODS: We investigated cerebrovascular reactivity in a mouse model of AD (APP-PS1; 5-6-month-old male subjects). We assessed the in vivo response to normoxic hypercapnia and in vitro FMD in isolated cerebral and mesenteric resistance arteries before and after endothelin receptor blockade (bosentan)...
November 17, 2023: Journal of Vascular Research
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