Concentrated solar power (CSP) is one of the few sustainable energy technologies that offers day-to-night energy storage. Recent development of the supercritical carbon dioxide (sCO2) Brayton cycle has made CSP a potentially cost-competitive energy source. However, as CSP plants are most efficient in desert regions, where there is high solar irradiance and low land cost, careful design of a dry cooling system is crucial to make CSP practical.
View Article and Find Full Text PDFMyasthenia gravis (MG) stands as a prototypical antibody-mediated autoimmune disease: it is dependent on T cells and characterized by the presence of autoantibodies targeting proteins located on the postsynaptic surface of skeletal muscle, known as the neuromuscular junction. Patients with MG exhibit a spectrum of weakness, ranging from limited ocular muscle involvement to life-threatening respiratory failure. Recent decades have witnessed substantial progress in understanding the underlying pathophysiology, leading to the delineation of distinct subcategories within MG, including MG linked to AChR or MuSK antibodies as well as age-based distinction, thymoma-associated, and immune checkpoint inhibitor-induced MG.
View Article and Find Full Text PDFThe role of immunosenescence, particularly the natural process of thymic involution during aging, is increasingly acknowledged as a factor contributing to the development of autoimmune diseases and cancer. Recently, a concern has been raised about deleterious consequences of the surgical removal of thymic tissue, including for patients who undergo thymectomy for myasthenia gravis (MG) or resection of a thymoma. This review adopts a multidisciplinary approach to scrutinize the evidence concerning the long-term risks of cancer and autoimmunity postthymectomy.
View Article and Find Full Text PDFBackground: Metabolic dysfunction associated steatotic liver disease (MASLD) has been linked to heart failure with preserved ejection fraction (HFpEF). We sought to understand association between individuals with amounts of liver adiposity greater than would be predicted by their body mass index (BMI) in order to understand whether this disproportionate liver fat (DLF) represents a proxy of metabolic risk shared between liver and heart disease.
Methods: We studied 2,932 participants in the Multi-Ethnic Study of Atherosclerosis (MESA) who received computed tomography (CT) measurements of hepatic attenuation.
Heart Fail Clin
April 2024
Already a challenging condition to define, adult congenital heart disease (ACHD) -associated heart failure (HF) often incorporates specific anatomies, including intracardiac and extracardiac shunts, which require rigorous diagnostic characterization and heighten the importance of clinicians proactively considering overall hemodynamic impacts of using specific therapies. The presence of elevated pulmonary vascular resistance dramatically increases the complexity of managing patients with ACHD-HF. Total circulatory management in patients with ACHD-HF requires input from multidisciplinary care teams and thoughtful and careful utilization of medical, interventional, and surgical approaches.
View Article and Find Full Text PDFObjective: High-dose prednisone use, lasting several months or longer, is the primary initial therapy for myasthenia gravis (MG). Upwards of a third of patients do not respond to treatment. Currently no biomarkers can predict clinical responsiveness to corticosteroid treatment.
View Article and Find Full Text PDFPercutaneous drains have provided a minimally invasive way to treat a wide range of disorders from abscess drainage to enteral feeding solutions to treating hydronephrosis. These drains suffer from a high rate of dislodgement of up to 30% resulting in emergency room visits, repeat hospitalizations, and catheter repositioning/replacement procedures, which incur significant morbidity and mortality. Using ex vivo and in vivo models, a force body diagram was utilized to determine the forces experienced by a drainage catheter during dislodgement events, and the individual components which contribute to drainage catheter securement were empirically collected.
View Article and Find Full Text PDFAntigen activated naïve B cells undergoing germinal center responses have distinct metabolic requirements.
View Article and Find Full Text PDFThe bicuspid aortic valve (BAV) is the most common congenital heart defect with an estimated prevalence of between 0.5% and 2% in the United States, representing up to 6.5 million individuals.
View Article and Find Full Text PDFBackground: Treatment of benign primary cardiac tumors involves surgical resection, but reported outcomes from multi-institutional or national databases are scarce. This study examines contemporary national outcomes following surgical resection of benign primary atrial and ventricular tumors.
Methods: The 2016-2018 Nationwide Readmissions Database was queried for all patients ≥18 years with a primary diagnosis of benign neoplasm of the heart who underwent resection of the atria, ventricles, or atrial/ventricular septum.
Background: During professional identity formation, medical students integrate their newly developing professional identities with their longstanding personal identities. Longitudinal mentorship has been shown to aid students in this process. Lack of clear relationship expectations among students and faculty is a barrier to effective longitudinal mentorship relationships.
View Article and Find Full Text PDFRett syndrome (RTT) is a severe neurodevelopmental disorder caused by loss of function of the X-linked methyl-CpG–binding protein 2 (). Several case studies report that gross motor function can be improved in children with RTT through treadmill walking, but whether the MeCP2-deficient motor circuit can support actual motor learning remains unclear. We used two-photon calcium imaging to simultaneously observe layer (L) 2/3 and L5a excitatory neuronal activity in the motor cortex (M1) while mice adapted to changing speeds on a computerized running wheel.
View Article and Find Full Text PDFThe bicuspid aortic valve (BAV) is a common and heterogeneous congenital heart abnormality that is often complicated by aortic stenosis. Although initially developed for tricuspid aortic valves (TAV), transcatheter aortic valve replacement (TAVR) devices are increasingly applied to the treatment of BAV stenosis. It is known that patient-device relationship between TAVR and BAV are not equivalent to those observed in TAV but the nature of these differences are not well understood.
View Article and Find Full Text PDFProblem: Mentorship is valuable to medical students undergoing professional identity formation. Many institutions lack infrastructure to facilitate the personalized mentoring that supports students' integration of new professional identities with their personal identities and values.
Approach: The authors developed a novel mentorship platform called Weave via a multistep, iterative design process, incorporating in-person and survey-based student and faculty feedback.
Myasthenia gravis (MG) is an autoimmune disease in which Abs target neuromuscular junction proteins, in particular the acetylcholine receptor. We previously identified the antiapoptotic protein survivin in the autoreactive B cells and plasma cells of MG patients. To further define the role of survivin in MG, we have assessed PBMCs from 29 patients with MG and 15 controls.
View Article and Find Full Text PDFThe thymus, a primary lymphoid organ, provides a complex environment essential for the generation of the T-cell repertoire. Thymic alterations occur during life either in the context of thymic involution upon aging or the pathophysiological context of Myasthenia Gravis (MG). These changes involve complicated regulatory networks, in which microRNAs (miRNAs) are key players.
View Article and Find Full Text PDFComplement-mediated damage to the neuromuscular junction (NMJ) is a key mechanism of pathology in myasthenia gravis (MG) and therapeutics inhibiting complement have shown evidence of efficacy in the treatment of MG. In this study, we describe the development of a subcutaneously administered N-acetylgalactosamine (GalNAc)-conjugated small interfering RNA (siRNA) targeting the C5 component of complement that silences C5 expression in the liver (ALN-CC5). Treatment of wild-type rodents with ALN-CC5 resulted in robust and durable suppression of liver C5 expression.
View Article and Find Full Text PDFBackground: A characteristic pathology of early onset myasthenia gravis is thymic hyperplasia with ectopic germinal centers (GC). However, the mechanisms that trigger and maintain thymic hyperplasia are poorly characterized. Dysregulation of small, non-coding microRNAs (miRNAs) and their target genes has been identified in the pathology of several autoimmune diseases.
View Article and Find Full Text PDFBecause of the failure of many promising therapeutics identified in preclinical evaluation, funding sources have established guidelines for increased rigor in animal evaluations. The myasthenia gravis (MG) community of scientists has developed guidelines for preclinical assessment for potential MG treatments. Here, we provide a focused summary of these recommendations and the role of complement in disease development in experimental models of MG.
View Article and Find Full Text PDFBiomarkers that assess treatment response for patients with the autoimmune disorder, myasthenia gravis (MG), have not been evaluated to a significant extent. We hypothesized the pro-inflammatory cytokine, osteopontin (OPN), may be associated with variability of response to glucocorticoids (GCs) in patients with MG. A cohort of 250 MG patients treated with standardized protocol of GCs was recruited, and plasma OPN and polymorphisms of its gene, secreted phosphoprotein 1 (), were evaluated.
View Article and Find Full Text PDFAn array of cytokines influences the pathogenesis of early onset myasthenia gravis (MG) and its animal model, experimental autoimmune myasthenia gravis (EAMG). Patients with MG, in particular those with more severe weakness, have elevations of the pro-inflammatory cytokine IL-17 in the blood. We assessed the role of IL-17A in autoimmunity by inducing EAMG in mice with knockout of IL-17 and found a reduction of EAMG severity, but not a complete ablation of disease.
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