FDA approves new orphan drug to treat pulmonary arterial hypertension

On December 21, the U.S. Food and Drug Administration approved Uptravi (selexipag) tablets to treat adults with pulmonary arterial hypertension (PAH), a chronic, progressive, and debilitating rare lung disease that can lead to death or the need for transplantation.
Source: Food and Drug Administration - Category: American Health Source Type: news

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Conclusions: In patients with FIP, PVR is a significant contributor of 6MWD, independently from the extent of fibrosis on HRCT. These results strengthen both the rationale to use 6MWD as endpoint in FIP and to target APH with specific therapies.Respiration
Source: Respiration - Category: Respiratory Medicine Source Type: research
We report that the disruption of excitation-contraction coupling contributes to impaired force generation in the mouse model of Sod1 deficiency. Briefly, we found a significant reduction in sarcoplasmic reticulum Ca2+ ATPase (SERCA) activity as well as reduced expression of proteins involved in calcium release and force generation. Another potential factor involved in EC uncoupling in Sod1-/- mice is oxidative damage to proteins involved in the contractile response. In summary, this study provides strong support for the coupling between increased oxidative stress and disruption of cellular excitation contraction mac...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
The first registry of patients with pulmonary arterial hypertension (PAH), established by the US National Heart, Lung, and Blood Institute (NHLBI) in 1981, reported a median survival of 2.8 years in the empirical therapy era.1 Shortly after bosentan was approved in the early 2000s, the French registry reported a one-year survival of 88% in patients with PAH,2 while the Chinese registry reported 1,3, and 5-year survivals of 68%, 38.9%, and 20.8% respectively, in patients with idiopathic and familial PAH who were treated with conventional therapies.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Invited Commentary Source Type: research
ConclusionsPatients with CS have similar post-transplant hemodynamics as patients without CS, without evidence of right ventricular dysfunction or pulmonary hypertension. Neither significant rejection nor recurrence of sarcoid in the allograft was observed in this cohort of patients with CS. Survival is similar between patients with CS and those without CS. Heart transplant is a viable strategy in selected patients with CS with excellent outcomes.RésuméIntroductionLa transplantation cardiaque orthotopique (TCO) est de plus en plus utilisée lors d’insuffisance cardiaque terminale liée &agra...
Source: Canadian Journal of Cardiology - Category: Cardiology Source Type: research
We presently forget 98% of everything we experience. That will go away in favor of perfect, controllable, configurable memory. Skills and knowledge will become commodities that can be purchased and installed. We will be able to feel exactly as we wish to feel at any given time. How we perceive the world will be mutable and subject to choice. How we think, the very fundamental basis of the mind, will also be mutable and subject to choice. We will merge with our machines, as Kurzweil puts it. The boundary between mind and computing device, between the individual and his or her tools, will blur. Over the course of the ...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
Registry data suggest that survival in pulmonary arterial hypertension (PAH) patients has improved from a median of 2.5 years from diagnosis to about 7 years in mostly prevalent cohorts,1,2 which is still dismal. There has long been a concern that initiation of therapy in PAH patients is insufficiently aggressive, given such poor survival. Until recently, data on the utility of combination therapy and the use of initial combination therapy in treatment-na ïve patients has been conflicting, uncontrolled, or nonexistent.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Original Clinical Science Source Type: research
Authors: Olland A, Falcoz PE, Massard G Abstract Lung transplantation has become an efficient life-saving treatment for patients with end stage respiratory disease. The increasing good outcome following lung transplantation may be explained by growing experience of transplant teams and availability of potent immunosuppressive drugs. Nevertheless, the latter carries an inherent risk for malignancy besides other common side effects such as systemic hypertension, diabetes and renal dysfunction. Malignancies occur in a smaller proportion of patients but explain for a large proportion of deaths following transplantation...
Source: Journal of Thoracic Disease - Category: Respiratory Medicine Tags: J Thorac Dis Source Type: research
CONCLUSION: The diagnostics and treatment of CTD-ILD and PH in patients with an underlying autoimmune disease requires an interdisciplinary approach. The effectiveness of antifibrotic treatment needs to be evaluated in the future. PMID: 30006857 [PubMed - as supplied by publisher]
Source: Zeitschrift fur Rheumatologie - Category: Rheumatology Authors: Tags: Z Rheumatol Source Type: research
Repair of Hemi-Truncus with "Irreversible" Pulmonary Hypertension. Ann Thorac Surg. 2018 Jul 13;: Authors: Sha JM, Cao Y, Xu SS Abstract Anomalous origin of the pulmonary artery from the ascending aorta (AOPA) can lead to congestive heart failure in infancy and with advancing age many patients will develop severe pulmonary hypertension. Surgical intervention had high mortality/morbidity risk if this happens. Strategies to manage these patients seem only limited to heart-lung transplantation or lung transplantation. Here, we successfully carried out surgical intervention to in an adult patien...
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: Ann Thorac Surg Source Type: research
Conclusion: Merging 3 haemodynamic variables into 1 variable, the RV index increased the prognostic power up to an independent risk factor. The RV index is easy to calculate and allows the construction of a nomogram for an individualized risk assessment.Respiration
Source: Respiration - Category: Respiratory Medicine Source Type: research
More News: American Health | Food and Drug Administration (FDA) | Hypertension | Lung Transplant | Transplants