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https://www.selleckchem.com/products/torin-1.html ps. Presence of schistocytes in peripheral blood smear supporting haemolysis is important for diagnosis and decision-making in paediatric haemolytic uraemic syndrome (HUS). High observer dependency and requirement of expertise for peripheral smear evaluation propels us to think of other modalities to overcome these issues. We envisage that newer techniques like automated fragmented red blood cell percentage (FRC %), whose role has been described in transplant associated HUS and thrombotic thrombocytopenic purpura, can serve the purpose. Twenty-eight children with HUS after excluding secondary causes were enrolled in this study. Blood samples were analysed for FRC% at admission, using SYSMEX XN-1000 (Japan) haematology analyser, and simultaneously, schistocytes in peripheral smear were reported by a single expert haematopathologist. Median age was 56months ranging from 2 to 140months. FRC% was elevated in 85.8% (n-24/28) with a mean of 4.56±3.1%. FRC% had a sensitivity of 95.4% (C.I 77.16% to 99.88%) in children who had FRC% >1.49% with an accuracy of 85.71% (C.I 67.33% to 95.97%). However, specificity was only 50% (C.I 11.81% to 88.19%) with a positive likelihood ratio of 1.91. Receiver-operator curve showed an AUC value of 0.841. We suggest automated FRC% as a rapid and highly sensitive index for screening of paediatric HUS; however, a peripheral blood film examination is a must in cases with count >2% to avoid false positives as the index has low specificity. 2% to avoid false positives as the index has low specificity.Venglustat is a small-molecule glucosylceramide synthase (GCS) inhibitor designed to reduce the production of glucosylceramide (GL-1) and thus is expected to substantially reduce formation of glucosylceramide-based glycosphingolipids. Because of its effect on glycosphingolipid formation, GCS inhibition has therapeutic potential across many disorders affecting glycosphingolipid metabolism. Therefore,

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