The GM2 gangliosidoses, Tay-Sachs disease and Sandhoff disease, are devastating neurodegenerative disorders caused by β-hexosaminidase A (HexA) deficiency. In the Sandhoff disease mouse model, rescue potential was severely reduced when HexA was introduced after disease onset. Here, we assess the effect of recombinant HexA and HexD3, a newly engineered mimetic of HexA optimized for the treatment of Tay-Sachs disease and Sandhoff disease.
View Article and Find Full Text PDFIndian J Thorac Cardiovasc Surg
May 2024
Unlabelled: Infective endocarditis surgical patients suffer from high rates of severe complications such as systemic inflammatory response, septic shock, and multi-organ failure leading to high mortality. Systemic inflammatory response based on cytokines as messengers plays an important role in these patients. The concept of intraoperative haemoadsorption has been proposed to remove such elevated cytokines in patients undergoing cardiac surgery for infective endocarditis.
View Article and Find Full Text PDFBackground: Extracorporeal blood purification has been widely used in intensive care medicine, nephrology, toxicology, and other fields. During the last decade, with the emergence of new adsorptive blood purification devices, hemoadsorption has been increasingly applied during CPB in cardiac surgery, for patients at different inflammatory risks, or for postoperative complications. Clinical evidence so far has not provided definite answers concerning this adjunctive treatment.
View Article and Find Full Text PDFIntraoperative antithrombotic drug removal by haemoadsorption is a novel strategy to reduce perioperative bleeding in patients on antithrombotic drugs undergoing cardiac surgery. The international STAR registry reports real-world clinical outcomes associated with this application. All patients underwent cardiac surgery before completing the recommended washout period.
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