A new analysis that reviewed data from 2 studies in patients with pulmonary arterial hypertension (PAH) has found a benefit to initiating selexipag therapy within 6 months of diagnosis. The study, published in ERJ Open Research, found reduced disease progression in newly diagnosed patients in the TRITON and GRIPHON clinical trials who were on active…
Researchers reported that a risk score developed based on 4 echocardiographic parameters may further help clinicians in stratifying mortality risk in patients with pulmonary arterial hypertension (PAH). Their study was published in Chest. Current mortality risk assessment tools in PAH rely on parameters that rarely include echocardiographic features. For example, the European Society of Cardiology…
Researchers determined that the minimal clinically important difference in 6-minute walk distance (6MWD) is around 33 meters for adults with pulmonary arterial hypertension (PAH). Their study is published in the American Journal of Respiratory and Critical Care Medicine. PAH is characterized by elevated pulmonary vascular resistance and right ventricular failure. Clinical trials that have led…
The presence of well-established common genetic variants as well as rare variants within the TERT and RTEL1 genes may significantly increase the risk of developing idiopathic pulmonary fibrosis (IPF), according to a recent study in the American Journal of Respiratory and Critical Care Medicine. This was the first whole-genome sequencing study that examined rare variant…
A new randomized controlled clinical trial (EXAFIP2) aiming to assess the efficacy of glucocorticoids during acute exacerbations of idiopathic pulmonary fibrosis (IPF) will begin in March 2023. The study will enroll 110 adults with IPF or suspected IPF who present with definite or suspected acute exacerbation. Participants randomized to the glucocorticoids group will receive 10…
Occupational exposure to asbestos alone is not associated with an increased risk of developing idiopathic pulmonary fibrosis (IPF), according to a study published in Occupational & Environmental Medicine. IPF is a progressive fibrotic lung disorder that impacts manual workers more than individuals in other job categories. Occupational exposure to asbestos has long been theorized to…
The Cystic Fibrosis Foundation will invest significantly in 2 investigational gene therapies for patients with cystic fibrosis (CF). The therapies aim to use novel nanoparticle technologies to deliver correct copies of CF transmembrane conductance regulator (CFTR) messenger RNA (mRNA) to lung cells so they can produce functional CFTR proteins. “Messenger RNA therapy has the potential…
Researchers presented the case study of a patient with sickle cell disease who later developed acute myeloid leukemia .
Pulmonary embolisms (PEs) in hospitalized children with sickle cell disease (SCD) might occur in 0.53% of the examined cases as well as those patients with higher disease severity, according to a new research article published in Pediatric Hematology and Oncology. For the purpose of this study, the researchers reviewed the Pediatric Health Information System to…
Researchers discovered that the PiZ variant in alpha-1 antitrypsin deficiency (AATD) is significantly higher in patients with bronchiectasis compared to the general population, according to a study published in Archivo de Bronconeumologia. Studies indicate that AATD is a potentially treatable cause of bronchiectasis. In terms of genetic prevalence, the PiZ variant is the most common…
