Tracleer (bosentan) / J&J, Roche 
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  • ||||||||||  Tracleer (bosentan) / J&J, Roche
    Journal:  High-Fidelity Drug Induced Liver Injury Screen Using Human PSC-derived Organoids. (Pubmed Central) -  Jul 24, 2021   
    Not yet recruiting --> Recruiting Liver organoid-based Toxicity screen (LoT) is a potential assay system for liver toxicology studies, facilitating compound optimization, mechanistic study, and precision medicine as well as drug screening applications.
  • ||||||||||  Tracleer (bosentan) / J&J, Roche
    Journal:  Endothelin-1 as a Mediator of Heme Oxygenase-1-Induced Stemness in Colorectal Cancer: Influence of p53. (Pubmed Central) -  Jul 4, 2021   
    Carbon monoxide (CO) activated the ECE-1/endothelin-1 (ET-1) pathway, which could account for the protumoral effects of HO-1 in p53 wild-type cells, as demonstrated after treatment with bosentan (an antagonist of both ETRA and ETRB endothelin-1 receptors)...However more research is warranted to confirm these results. Patients carrying tumors with a high expression of both HO-1 and ECE-1 and a non-wild-type p53 should be considered for HO-1 based-therapies instead of ET-1 antagonists-based ones.
  • ||||||||||  Tracleer (bosentan) / J&J, Roche
    Review, Journal:  Congenital diaphragmatic hernia: a narrative review of controversies in neonatal management. (Pubmed Central) -  Jul 1, 2021   
    Additionally, the results of the many studies currently within the literature that have investigated differing aspect of care (i.e., inhaled nitric oxide, ventilator type, timing of repair, role of extracorporeal membrane oxygenation, etc.) are difficult to interpret due to the small numbers investigated, the varying degree of physiologic compromise, and the contrasting care that exists between institutions. The aim of this paper is to review areas of controversy in the care of these complex kids, mainly: the use of fraction of inspired oxygen, surfactant therapy, gentle ventilation, mode of ventilation, medical management of pulmonary hypertension (inhaled nitric oxide, sildenafil, milrinone, bosentan, prostaglandins), the utilization of extracorporeal membrane oxygenation, and the timing of surgical repair.
  • ||||||||||  Tracleer (bosentan) / J&J, Roche
    Journal:  The Influence of Bosentan on MicroRNA-27a/PPARγ/ET-1 Signaling Pathway in Pulmonary Artery Hypertension. (Pubmed Central) -  Jun 26, 2021   
    Our results demonstrated that during the pathophysiology of PAH, miR-27a, PPARγ, and ET-1 were cross-inhibited, which indicated that the miR-27a/PPARγ/ET-1 signaling pathway was dysregulated; in addition, BST could competitively bind to ET-1 receptors and inhibit the miR-27a/PPARγ/ET-1 signaling pathway, thereby delaying the proliferation of PASMCs and affecting the development of PAH. Our results give a new understanding of the pathogenesis and treatment of PAH and provide more reliable evidence for the application of BST in the treatment of PAH in the clinic.
  • ||||||||||  ritonavir / Generic mfg.
    Preclinical, Journal:  In vitro assessment of farnesoid X receptor antagonism to predict drug-induced liver injury risk. (Pubmed Central) -  Jun 22, 2021   
    In addition, we have identified novel FXR antagonistic effects of well-studied hepatotoxic drugs, including bosentan, tolcapone and ritonavir. In conclusion, this work represents a comprehensive evaluation of FXR antagonism in the context of DILI, including its overall predictivity and challenges associated with detecting this phenomenon in vitro.
  • ||||||||||  Ventavis (iloprost) / Bayer, J&J, University of Copenhagen
    Clinical, Review, Journal:  Evolution of randomized, controlled studies of medical therapy in chronic thromboembolic pulmonary hypertension. (Pubmed Central) -  Jun 10, 2021   
    Studies of medical therapies for CTEPH have evolved since Aerosolized Iloprost Randomized (AIR), the first randomized, controlled study of a PH-targeted therapy (inhaled iloprost) to include patients with CTEPH...The 16-week Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase-Stimulator Study 1 (CHEST-1) study was the first to operationalize these learnings, demonstrating a significant mean improvement in 6-minute walk distance (+46 m) and improvements in hemodynamic endpoints with riociguat versus placebo...Data on combinations of macitentan with phosphodiesterase type 5 inhibitors or oral prostanoids are available from MERIT, the first study to allow such regimens...Studies are also needed for multimodality treatment, including medical therapy plus BPA, and medical therapy as a bridge to PEA in selected operable patients. To address these issues and improve patient outcomes, it is vital that we learn from current studies to improve future trial design.
  • ||||||||||  Ventavis (iloprost ) / Bayer, J&J, University of Copenhagen
    [VIRTUAL] RAYNAUD’S PHENOMENON IN A SINGLE CENTER COHORT OF TURKISH CHILDREN (Posters Viewing) -  May 21, 2021 - Abstract #EULAR2021EULAR_3664;    
    In patients with secondary RP, the symptoms. It has been shown that in patients with secondary RP, symptoms begin at a younger age and the ANA positivity and abnormal nailfold capillaries correlate.
  • ||||||||||  Tracleer (bosentan) / J&J, Roche
    Journal:  Endotheliitis, endothelin, and endothelin receptor blockers in COVID-19. (Pubmed Central) -  Apr 28, 2021   
    Endothelin receptor blockers, such as bosentan and sitaxentan, can pave a path ahead in the realm of COVID-19 therapies. These agents have a potential role against COVID-19 and should be studied in research trials to determine their efficacy in treatment of this severe disease.
  • ||||||||||  Tracleer (bosentan) / J&J, Roche
    Clinical, Journal:  Update on pediatric pulmonary arterial hypertension. (Pubmed Central) -  Apr 24, 2021   
    In recent years, advanced imaging and clinical monitoring have allowed improved risk stratification of pulmonary hypertension patients. New therapies, approved in adults and used off-label in pediatric patients, have led to improved outcomes for affected children.
  • ||||||||||  Tracleer (bosentan) / J&J, Roche
    Trial completion date, Trial primary completion date:  BOSRET: Safety of Bosentan in Type II Diabetic Patients (clinicaltrials.gov) -  Apr 22, 2021   
    P1,  N=22, Recruiting, 
    New therapies, approved in adults and used off-label in pediatric patients, have led to improved outcomes for affected children. Trial completion date: Dec 2020 --> Dec 2022 | Trial primary completion date: Dec 2020 --> Dec 2022
  • ||||||||||  Tracleer (bosentan) / J&J, Roche
    Trial completion date, Trial primary completion date:  MoD: Mono vs. Dual Therapy for Pediatric Pulmonary Arterial Hypertension (clinicaltrials.gov) -  Apr 22, 2021   
    P3,  N=100, Not yet recruiting, 
    Trial completion date: Dec 2020 --> Dec 2022 | Trial primary completion date: Dec 2020 --> Dec 2022 Trial completion date: Mar 2026 --> Jul 2026 | Trial primary completion date: Mar 2026 --> Jul 2026
  • ||||||||||  [VIRTUAL] EFFECTS OF FACE-TO-FACE EDUCATIONAL NURSING SUPPORT ON PATIENT COMPLIANCE WITH ORAL PAH THERAPIES () -  Apr 11, 2021 - Abstract #AMCP2021AMCP_128;    
    Patients receiving oral PAH therapies in an educational nursing program demonstrated a positive effect on overall medication compliance as demonstrated by a statistically significant improvement in MPR and therapy persistence. While our study compared two different PAH therapy classes, a multidisciplinary patient support approach contributed to improved patient outcomes.
  • ||||||||||  Tracleer (bosentan) / J&J, Roche
    Journal:  Modified karaya gum colloidal particles for the management of systemic hypertension. (Pubmed Central) -  Apr 10, 2021   
    Pre-clinical testing via oral route demonstrated that the crosslinked colloidal particles could effectively control the systemic hypertension over a period of 12 h. Hence, bosentan-loaded self-assembled colloidal particles may advance the management of systemic hypertension.
  • ||||||||||  Tracleer (bosentan) / J&J, Roche
    Journal:  Oral drugs used to treat persistent pulmonary hypertension of the newborn. (Pubmed Central) -  Apr 7, 2021   
    Accordingly, bosentan, sildenafil and tadalafil remain off-label in clinical use. Therefore, more well-designed randomized controlled trials with long-term follow-up and pharmacometrics studies are needed to demonstrate the efficacy, safety and pharmacokinetics of bosentan, sildenafil and tadalafil in PPHN.
  • ||||||||||  Ventavis (iloprost ) / Bayer, J&J, University of Copenhagen
    [VIRTUAL] INTERNATIONAL MULTICENTRIC PROSPECTIVE STUDY ON PREGNANCY IN SYSTEMIC SCLEROSIS (IMPRESS-2) (Room 12) -  Apr 6, 2021 - Abstract #EULAR2021EULAR_1415;    
    SSc pregnancies have generally a favorable obstetric/pediatric outcome, albeit at a higher risk of gestational hypertension, pre-eclampsia, fetal growth restriction, prematurity, delivery of SGA newborn and requirement of neonatal-ICU. Pregnancy does not impact on SSc course, although SRC during late pregnancy and pre-eclampsia might be hardly discriminated.
  • ||||||||||  Ventavis (iloprost ) / Bayer, J&J, University of Copenhagen
    Clinical, Review, Combination therapy:  Advantages and limitations of initial combination therapy in pulmonary arterial hypertension patients in Russia (Pubmed Central) -  Mar 23, 2021   
    Some beneficial pharmacological effects due to the synergy between ambrisentan plus riociguat, and inhaled iloprost plus sildenafil appear to be interesting and require further clinical confirmation. Other initial combinations of PAH-specific agents require large-scale clinical trials as well.
  • ||||||||||  Tracleer (bosentan) / J&J, Roche
    Trial completion date, Trial primary completion date:  Gemcitabine, Nab-Paclitaxel, and Bosentan for the Treatment of Unresectable Pancreatic Cancer (clinicaltrials.gov) -  Mar 15, 2021   
    P1,  N=21, Not yet recruiting, 
    Other initial combinations of PAH-specific agents require large-scale clinical trials as well. Trial completion date: Dec 2021 --> Dec 2022 | Trial primary completion date: Dec 2021 --> Dec 2022
  • ||||||||||  Tracleer (bosentan) / J&J, Roche, VentaProst (epoprostenol inhalation) / Aerogen Pharma
    [VIRTUAL] Novel Filamin A Mutation Causing Advanced Infant Pulmonary Disease () -  Mar 14, 2021 - Abstract #ATS2021ATS_4608;    
    Lung explant examination confirmed a marked alveolar growth abnormality that differed from the alveolar destruction defining emphysema. This work adds to our understanding of lung disease associated with FLNA mutations.
  • ||||||||||  Tracleer (bosentan) / J&J, Roche
    [VIRTUAL] A Rare Case of Pericardial Effusion in a Pediatric Patient () -  Mar 14, 2021 - Abstract #ATS2021ATS_2631;    
    Conclusion Pericardial effusion is common in adults with OSA but has not been described in children. This patient’s underlying sleep issues may have contributed her cardiac pathology.