Researchers from the US and the UK have identified plasma biomarkers that are unique to and shared between alpha-1 antitrypsin deficiency (AATD) and chronic obstructive pulmonary disease (COPD). The researchers also generated a protein score that is strongly associated with emphysema in patients with these 2 conditions. Finally, they identified unique biomarkers associated with diffusion…
Researchers from China have identified ubiquitin-specific protease 13 (USP13) as a regulator of autophagy and age-related lung fibrosis in a mouse model. The results of their study were recently published in the American Journal of Respiratory Cell and Molecular Biology and contribute to current knowledge on the pathogenesis of idiopathic pulmonary fibrosis (IPF). “We hypothesize…
The calcium release-activated calcium channel protein 1 (Orai1) could be a potential therapeutic target for pulmonary arterial hypertension (PAH), according to a new study published in Circulation Research. “Pharmacological inhibition of Orai1 in vivo improves pulmonary vascular remodeling, right ventricular fibrosis, and hypertrophy and reduces the development of experimental PAH,” the study’s authors said. Masson…
The secreted glycoprotein neuroblastoma suppressor of tumorigenicity 1 (NBL1) worsens flow-associated pulmonary arterial hypertension (PAH) by inducing endothelial to mesenchymal transition via the transforming growth factor beta (TGF-β)/bone morphogenetic protein (BMP) signaling pathway. This is according to a new study published in the American Journal of Respiratory Cell and Molecular Biology. These findings suggest that…
I often consider whether talking about alpha-1 antitrypsin deficiency (AATD) with family members is a good idea before I open my mouth.
Researchers have reported a case in which a patient’s exacerbations of alpha-1 antitrypsin deficiency were successfully reduced with the use of augmentation therapy. The case report, published in Respiratory Medicine Case Reports, noted that the patient also had bronchiectasis and suggested that clinicians should consider AATD as a possible cause of this condition. “Whilst there…
Researchers from the UK found that restoration of sinus rhythm was associated with functional improvement and superior survival in patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). They suggested that electrical cardioversion (DCCV) could be an effective strategy to restore normal rhythm. “DCCV is generally safe and more effective at restoring…
Potential biomarkers of pulmonary arterial hypertension (PAH) and congenital heart disease (CHD), which can be used alone or in combination to distinguish patients with PAH-CHD from those with CHD, have been identified with the use of nuclear magnetic resonance (NMR)-based metabolomic analyses, according to the results of a recently published study in Metabolites. Recognizing that…
Researchers will conduct a trial to study the efficacy of ARO-MMP7 inhalation solution in both healthy subjects and patients with idiopathic pulmonary fibrosis (IPF). The sponsor of this study is Arrowhead Pharmaceuticals. ARO-MMP7 is the company’s experimental RNA interference treatment that is designed to lower the expression of matrix metalloproteinase 7 (MMP7). MMP7 is a…
The combination of the small molecule ELX-02 and ivacaftor (Kalydeco®) was well tolerated by cystic fibrosis (CF) patients with class I mutations, but failed to achieve efficacy endpoints of a phase 2 trial. Efficacy endpoints included changes from baseline in sweat chloride concentration and percent forced expiratory volume. “We are disappointed that ELX-02 failed to…
