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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Authors: Naranjo M, Lo KB, Mezue K, Rangaswami J Abstract Pulmonary hypertension is not uncommon in patients with renal disease and vice versa; therefore, influences treatments and outcomes. There is a large body of literature on pulmonary hypertension in patients with kidney disease, its prognostic implications, economic burden, and management strategies. However, the converse, namely the hemodynamic effects of pulmonary hypertension on kidney function (acute and chronic kidney injury) is less studied and described. There is also increasing interest in the effects of pulmonary hypertension on kidney transplant out...
Source: Current Cardiology Reviews - Category: Cardiology Tags: Curr Cardiol Rev Source Type: research
This article, unfortunately paywalled, is interesting to note as a mark of the now increasingly energetic expansion of commercial efforts in longevity science. David Sinclair has been building a private equity company to work in many areas relevant to this present generation of commercial longevity science; while I'm not sold on his primary research interests as the basis for meaningful treatments for aging, he is diversifying considerably here, including into senolytics, the clearance of senescent cells demonstrated to produce rejuvenation in animal studies. This sort of approach to business mixes aspects of investing and...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
The objective of this review is to describe the potential applications of POCUS in end-stage renal disease patients (ESRD). Recent findings With basic training, the clinician can perform pulmonary, vascular, cardiac, and abdominal POCUS at the bedside of ESRD patients. Pulmonary ultrasound can be used to quantify pulmonary congestion and for the differential diagnosis of dyspnea. Ultrasound of the inferior vena cava combined with simple cardiac ultrasound can be used to promptly investigate the mechanism of hemodynamic instability. Vascular ultrasound can be used for troubleshooting of arteriovenous fistula problems and...
Source: Current Opinion in Nephrology and Hypertension - Category: Urology & Nephrology Tags: DIALYSIS AND TRANSPLANTATION: Edited by J. Kevin Tucker and Anil Chandraker Source Type: research
Purpose of review The purpose of this review is to highlight recent data regarding feasibility and outcomes following lung transplantation for patients with systemic sclerosis related pulmonary disease as well as to emphasize areas of uncertainly in need of further study. We include a description of our centre's approach to lung transplant evaluation and posttransplant management in this complex patient population. Recent findings Historical data have demonstrated that patients with scleroderma have an increased risk of complications following lung transplantation owing to the multisystem nature of disease, particular...
Source: Current Opinion in Rheumatology - Category: Rheumatology Tags: RAYNAUD PHENOMENON, SCLERODERMA, OVERLAP SYNDROMES AND OTHER FIBROSING SYNDROMES: Edited by John Varga Source Type: research
This study assessed the prevalence of syncope in pediatric PAH and examined its clinical, hemodynamic, and prognostic importance. This retrospective study assessed clinical data, including syncope status, from 78 children (37 girls) with idiopathic and heritable PAH (median age at diagnosis, 11  years). Patients were classified as syncopal or non-syncopal, and clinical data from the two groups were compared. The primary outcome was a composite of lung transplantation and cardiac mortality. Overall, 31 (38%) children had a history of syncope at presentation. Median age at diagnosis, sex ra tio, brain natriuretic peptid...
Source: Pediatric Cardiology - Category: Cardiology Source Type: research
AbstractLV is a pressure-generating pump which endures pressure overload, while RV is a flow-generating pump intolerant of pressure overload. Therefore, RV pump function (but not RV myocardial contractility) can easily fail in face of severe pulmonary arterial hypertension (PH) because of increased afterload. Available indexes of RV function are load dependent and incapable of accurately reflecting RV myocardial contractility. Animal RV in which myocardium is damaged extensively by either soldering iron or coronary occlusion can work well without causing systemic congestion or decreased SV. In clinical settings, evaluation...
Source: Journal of Echocardiography - Category: Cardiology Source Type: research
ConclusionsOur data indicate a trend toward improved survival over time of CTD-PAH and CTEPH patients treated at a Portuguese referral PH center. Earlier diagnosis, increasing use of parenteral prostanoids, and surgical treatment may further improve PH prognosis.ResumoIntroduçãoEste estudo tem como objetivo avaliar a sobrevivência a longo prazo em doentes com hipertensão arterial pulmonar (PAH) e hipertensão pulmonar tromboembólica crónica (CTEPH) seguidos num centro de tratamento.MétodosDoentes diagnosticados com PAH ou CTEPH entre janeiro 2005 e dezembro 2016 foram i...
Source: Revista Portuguesa de Cardiologia - Category: Cardiology Source Type: research
Patients with pulmonary hypertension (PH) due to chronic lung disease (Group 3 PH) have poor long-term outcomes. However, predictors of survival in Group 3 PH are not well described.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Original Clinical Science Source Type: research
Pulmonary compromise has been shown to significantly contribute to nonrelapse mortality after hematopoietic stem cell transplantation (HSCT) [1-4]. The etiology of these pulmonary complications includes intrapulmonary causes of fibrosis and toxicities from treatment regimens or infections (ie, restrictive lung disease), graft-versus-host disease (GVHD) or bronchiolitis obliterans syndrome (BOS) (ie, obstructive lung disease), and vascular abnormalities (eg, pulmonary hypertension), as well as extrapulmonary causes, such as weakness or sclerodermatous GVHD.
Source: Biology of Blood and Marrow Transplantation - Category: Hematology Authors: Source Type: research
The oral PGl2 receptor (IP) agonist, selexipag, has been shown in a long-term trial to delay disease progression in patients with pulmonary arterial hypertension (PAH). Transition to selexipag in patients treated with more burdensome inhaled prostacyclin-pathway targeting therapies may be considered by patients and physicians. The phase 3b, prospective, open-label TRANSIT-1 study evaluated the safety and tolerability of transition from inhaled treprostinil to oral selexipag.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
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