Riociguat treatment in patients with chronic thromboembolic pulmonary hypertension: Final safety data from the EXPERT registry

Objective: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following Phase

Prospects of Low Trophic Marine Aquaculture Contributing to Food Security in a Net Zero-Carbon World

To limit compromising the integrity of the planet, a shift is needed towards food production with low environmental impacts and low carbon footprint. How to put such transformative change towards sustainable food production whilst ensuring food security into practice remains a challenge and will require transdisciplinary approaches. Combining expertise from natural- and social sciences as well as industry perspectives, an alternative vision for the future in the marine realm is proposed. This vision includes moving towards aquaculture mainly of low trophic marine (LTM) species. Such shift may enable a blue transformation that can support a sustainable blue economy. It includes a whole new perspective and proactive development of policy-making which considers, among others, the context-specific nature of allocation of marine space and societal acceptance of new developments, over and above the decarbonization of food production, vis á vis reducing regulatory barriers for the industry for LTM whilst acknowledging the complexities of upscaling and outscaling. This needs to be supported by transdisciplinary research co-produced with consumers and wider public, as a blue transformation towards accelerating LTM aquaculture opportunities in a net zero-carbon world can only occur by considering the demands of society

Impact of elexacaftor/tezacaftor/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF RegistryResearch in context

Summary: Background: Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) improves multiple clinical outcomes in people with cystic fibrosis (pwCF) with at least one F508del allele. This study evaluated the real-world impact of ETI on lung function, nutritional status, pulmonary exacerbation frequency, and sweat chloride concentrations in a large group of pwCF. Methods: This observational cohort study used data from the German CF Registry for pwCF who received ETI therapy and were followed up for a period of 12 months. Findings: The study included 2645 pwCF from 67 centres in Germany (mean age 28.0 ± 11.5 years). Over the first year after ETI was initiated, percent predicted forced expiratory volume in 1 s (ppFEV1) increased by 11.3% (95% confidence interval [CI] 10.8–11.8, p < 0.0001), body mass index (BMI) z-score increased by 0.3 (95% CI 0.3–0.4, p < 0.0001) in individuals aged 12 to <18 years and BMI in adults increased by 1.4 kg/m2 (95% CI 1.3–1.4, p < 0.0001), pulmonary exacerbations decreased by 75.9% (p < 0.0001) and mean sweat chloride concentration decreased by 50.9 mmol/L (95% CI –52.6, −49.3, p < 0.0001). Improvements in ppFEV1 over the first year of therapy were greater in pwCF who had not previously received cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy (12.6% [95% CI 11.9–13.4] vs. 9.7% [95% CI 9.0–10.5] in those with prior CFTR modulator treatment. Interpretation: These real-world data are consistent with the findings of randomised clinical trials, and support the use of ETI as a highly effective treatment option for pwCF who have at least one F508del allele. Funding: None