Deficiency of an enzyme called granzyme B leads to deposits of calcium in pulmonary blood vessels, contributing to the defects found in hypoxic pulmonary arterial hypertension (PAH), an animal study suggests. The study, “Granzyme B deficiency promotes osteoblastic differentiation and calcification of vascular smooth muscle cells in…
Family and friends caring for people with rare diseases face emotional, financial, physical, and social burnout, partly because outside sources of support are limited, the first-of-its-kind U.S. survey shows. Global Genes and the National Alliance for Caregiving released the findings, which Greenwald & Associates conducted. It covered 1,406 family members caring…
The National Institutes of Health (NIH) awarded a $2.8 million Fast Track Small Business Innovation Research (SBIR) grant to PhaseBio Pharmaceuticals to advance the clinical development of PB1046, the first sustained-release analogue of the natural vasoactive intestinal peptide (VIP), in patients with pulmonary arterial hypertension (PAH). In…
Reata Pharmaceuticals announced that pulmonary arterial hypertension (PAH) patients treated with bardoxolone methyl showed significant improvements in kidney function that were sustained for two years without adverse outcomes. The results from the long-term follow-up of the Phase 2 LARIAT study (NCT02036970) showing a two-year improvement in kidney…
Non-invasive Doppler echocardiography does as good a job of assessing high blood pressure in lung arteries as the current invasive standard of right heart catheterization, a pulmonary hypertension study suggests. And doctors can use the results from either method to predict PH patients’ outcomes, researchers said. The team published their findings in…
Diagnosing and managing pulmonary hypertension in premature American infants with bronchopulmonary dysplasia vary a lot from doctor to doctor, a survey of 306 neonatologists indicates. The study, “Practices surrounding pulmonary hypertension and bronchopulmonary dysplasia amongst neonatologists caring for premature infants,” was published in the Journal of Perinatology.
A tool for estimating blood pressure on the left side of the heart is unable to predict the long-term survival of pulmonary hypertension patients taking Remodulin, a study reports. The tool, pulmonary capillary wedge pressure, or PCWP, is used to estimate blood pressure in the left atrium. United Therapeutics makes …
Using a multi-category index to assess pulmonary hypertension patients’ response to Adempas (riociguat) can help doctors predict their survival, a study reports. REVEAL risk scores can also help doctors predict patients’ progression-free survival — or the length of time it takes for their disease to worsen, researchers said. The study, which…
Echocardiograms can identify infants at risk of developing pulmonary hypertension, especially babies who are underweight, a German study reports. Early treatment can eliminate PH in many newborns, the study added. The research, in the journal Neonatology, is titled “An Echocardiographic Screening Program Helps to Identify Pulmonary Hypertension…
A study in mice offers insight into why women are more susceptible to developing pulmonary hypertension than men. It suggests that genes in the Y chromosome – which confers the male sex – may protect against the development of pulmonary hypertension. The finding suggests that differences in susceptibility to the…
Preclinical work supports the potential of sotatercept to be a first-in-class disease-modifying therapy for pulmonary arterial hypertension (PAH) and to moving the investigational therapy into a Phase 2 trial in patients, Acceleron Pharma announced. Sotatercept is a trap molecule for members of the transforming growth factor-beta (TGF-beta) superfamily. A growing body of evidence…
A compound that scientists have been examining as a cancer therapy reduced blood pressure in pulmonary hypertension patients’ lungs, according to a clinical trial. Researchers tested the therapy, dichloroacetic acid, in 20 patients with pulmonary arterial hypertension, or PAH. Although all responded, some with particular gene mutations failed to respond as well,…
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