D., Igor Adzerikho, M medication .D., Richard N. Channick, M.D., Marion Delcroix, M.D.D., Hossein-Ardeschir Ghofrani, M.D., Pavel Jansa, M.D., Zhi-Cheng Jing, M.D., Franck-Olivier Le Brun, M.Sc., Sanjay Mehta, M.D., Camilla M. Mittelholzer, Ph.D.D., B.K.S. Sastry, M.D., Olivier Sitbon, M.D.D., Adam Torbicki, M.D., Xiaofeng Zeng, M.D., Lewis J. Rubin, M.D.D. For the SERAPHIN Investigators: Macitentan and Morbidity and Mortality in Pulmonary Arterial Hypertension Pulmonary arterial hypertension, a serious disease characterized by a sustained elevation of pulmonary vascular resistance, potential clients to right heart failing and death ultimately.1 Disease progression occurs regardless of the availability of drugs that are particular for the disorder.2 Endothelin-receptor antagonists, phosphodiesterase type 5 inhibitors, and prostacyclin and its own analogues have been approved for the treating pulmonary arterial hypertension and adopted clinically on the basis of short-term trials which have shown improvements in exercise capability as measured by the distance walked in 6 minutes.3-10 However, current guidelines suggest that the principal end point in phase 3 trials of fresh remedies for pulmonary arterial hypertension ought to be morbidity and mortality.11-13 The dual endothelin-receptor antagonist macitentan was developed by modifying the structure of bosentan to increase efficacy and safety.14 Macitentan is characterized by sustained receptor binding and improved cells penetration.15,16 In the Study with an Endothelin Receptor Antagonist in Pulmonary Arterial Hypertension to Improve Clinical Outcome , we investigated whether long-term treatment with macitentan decreases morbidity and mortality among sufferers with pulmonary arterial hypertension.
These data claim that imetelstat may possess a selective inhibitory effect on the development of the neoplastic clone or clones that travel essential thrombocythemia. Although all of the patients had hematologic responses, there have been subsequent fluctuations above normal platelet levels in all patients. Once an initial response was achieved, individuals with platelet counts that were not managed by maintenance dosing for at least 8 weeks were considered to have a loss of response. The reported full hematologic responses were durable but required ongoing imetelstat therapy; interpatient variability was noted in the regularity of dosing necessary to manage platelet amounts .