Excessive vasoconstriction and excessive cellular proliferation play important roles in the pathogenesis of pulmonary arterial hypertension (PAH). Endothelin-1 (ET-1) is a potent vasoconstrictor that also promotes the proliferation of pulmonary artery smooth muscle cells. Patients with PAH have elevated levels of plasma ET-1, which correlate with disease severity and prognosis. Furthermore, the clearance of ET-1…
I live with pulmonary arterial hypertension (PAH), a progressive rare disease that is treated by addressing the symptoms only and not the disease.
Monotherapy with rodatristat ethyl reduces pulmonary vascular occlusions, according to results from a preclinical study in a rat model of pulmonary arterial hypertension (PAH). Treatment with a combination of rodatristat ethyl and ambrisentan, a type-A endothelin receptor antagonist, led to a synergistic benefit in reducing occlusions. They also reduced mean pulmonary arterial pressure. These results…
Researchers have discovered that serum Krebs von den Lungen-6 (KL-6) level may be a useful biomarker of disease progression in idiopathic pulmonary fibrosis (IPF), as published in Scientific Reports. IPF is a progressive fibrosing interstitial pneumonia that carries a poor prognosis, with a median survival of only 3 to 5 years. Currently, physiological parameters obtained…
The underdiagnosis and consequent undertreatment of sleep-disordered breathing in the CF population can compromise patients’ overall outcomes.
Researchers reported that there was still a disparity between objective and patient-based measures of chronic rhinosinusitis in individuals with cystic fibrosis (CF) who were treated with highly effective modulator therapy, and published their findings in the International Forum of Allergy & Rhinology. One of the keys to boosting the quality of life in patients with…
Transient elastography seems to be more useful than ultrasonography in identifying liver disease related to alpha-1 antitrypsin deficiency (AATD), according to a study presented at the American Thoracic Society (ATS) 2022 International Conference. The authors of the study said that larger prospective studies are needed to establish the use of the technique in staging liver…
Aytu BioPharma has developed an endotracheal respiratory catheter which its CEO says has “tremendous potential” in IPF, PAH, and other respiratory diseases.
Many people with alpha-1 antitrypsin deficiency (AATD) would agree they need some sort of support during humid days.
Larry Luxner, senior correspondent for Rare Disease Advisor, interviews Josh Disbrow, CEO of Colorado-based Aytu BioPharma.
