RESUMEN
Background: Lower extremity peripheral artery disease (LE-PAD) has been linked to unfavorable cardiovascular outcomes. The impact of potentially undiagnosed LE-PAD, suspected by abnormal ankle-brachial index (ABI), on the survival of sepsis patients admitted to the intensive care unit (ICU) remains uncertain. Methods: We conducted a prospective cohort study and recruited adult patients admitted to the ICU with a primary diagnosis of sepsis (defined by a quick Sepsis-Related Organ Failure Assessment score of ≥ 2) between November 23, 2017 and July 22, 2018. ABI measurements were obtained within 24 hours of admission. The study compared the 30-day and 1-year all-cause mortality rates as well as the incidence of major adverse cardiovascular events (MACEs) between the groups with normal and abnormal ABI values. Results: Of the 102 sepsis patients admitted to the ICU, 38 (37%) were diagnosed with LE-PAD based on their ABI measurements. The overall 30-day mortality rate was 30.0% in patients with LE-PAD and 25.8% in those with normal ABI (p = 0.56). At 1 year, the overall mortality rate was 52.6% in the patients with abnormal ABI and 40.6% in those with normal ABI (p = 0.24). Additionally, the incidence of MACEs was significantly higher in the patients with abnormal ABI compared to those with normal ABI at 1-year follow-up (21.1% vs. 3.1%, respectively; p = 0.003). Conclusions: The patients with abnormal ABI had a higher incidence of MACEs within one year following hospital discharge. Future studies are needed to improve cardiovascular outcomes among sepsis survivors (ClinicalTrials.gov number, NCT03372330).
RESUMEN
As an X-linked inherited lysosomal storage disease that is caused by α-galactosidase A gene variants resulting in progressive accumulation of pathogenic glycosphingolipid (Gb3) accumulation in multiple tissues and organs, Fabry disease (FD) can be classified into classic or late-onset phenotypes. In classic phenotype patients, α-galactosidase A activity is absent or severely reduced, resulting in a more progressive disease course with multi-systemic involvement. Conversely, late-onset phenotype, often with missense variants (e.g., IVS4+919G>A) in Taiwan, may present with a more chronic clinical course with predominant cardiac involvement (cardiac subtype), as they tend to have residual enzyme activity, remaining asymptomatic or clinically silent during childhood and adolescence. In either form, cardiac hypertrophy remains the most common feature of cardiac involvement, potentially leading to myocardial fibrosis, arrhythmias, and heart failure. Diagnosis is established through α-galactosidase enzyme activity assessment or biomarker analyisis (globotriaosylsphingosine, Lyso-Gb3), advanced imaging modalities (echocardiography and cardiac magnetic resonance imaging), and genotyping to differentiate FD from other cardiomyopathy. Successful therapeutic response relies on early recognition and by disease awareness from typical features in classic phenotype and cardiac red flags in cardiac variants for timely therapeutic interventions. Recent advances in pharmacological approach including enzyme replacement therapy (agalsidase alfa or beta), oral chaperone therapy (migalastat), and substrate reduction therapy (venglustat) aim to prevent from irreversible organ damage. Genotype- and gender-based monitoring of treatment effects through biomarker (Lyso-Gb3), renal assessment, and cardiac responses using advanced imaging modalities are key steps to optimizing patient care in FD.
RESUMEN
Blue-tailed forest hawk dragonfly (Orthetrum triangulare) wings, covered with inclined conical structures, are studied for their high transparency and low reflectance for large viewing angles. However, limited by existing technologies, the exquisite inclined structures are not replicated easily or applied adequately. Here, we combine a shear-induced self-assembly approach and a colloidal lithography technology to create omnidirectional antireflection structures that are inspired by dragonfly wings. Nonclose-packed colloid crystals are spin-coated and serve as structural templates in a plasma etching procedure to pattern subwavelength inclined conical structures directly on shape memory polymer-coated substrates. The dependence of the antireflection functionality on the shape and inclination of conical structures is systematically investigated in this research. Compared with a featureless substrate, the structure-covered substrate can display an approximately 8% higher average transmittance in the visible wavelength range at normal incidence and even approximately 23% higher average transmittance as the incident angle increases to 75°. Moreover, the reconfigurable structures composed of shape memory polymers can be repeatedly deformed and recovered as a result of external stimuli at ambient conditions, and the corresponding broadband omnidirectional antireflection functionality is therefore reversibly erased and restored.