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Use of complement C5-inhibitor eculizumab in patients with infection-associated hemolytic uremic syndrome - a case-series report.
BMC Pediatr
March 2025
Institute of Pediatrics, Faculty of Medicine, University of Debrecen, Nagyerdei Krt 98, 4028, Debrecen, Hungary.
Background: Hemolytic uremic syndrome (HUS), characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury (AKI), remains a leading cause of pediatric AKI. The complement system has a crucial role in the pathogenesis of atypical hemolytic uremic syndrome (aHUS) and eculizumab (ECZ) was approved as standard of care for its treatment. The two widely characterized forms of infection-associated HUS are Shiga toxin-producing E.
View Article and Find Full Text PDFThe function of T cells in immune thrombocytopenia.
Front Immunol
March 2025
Department of Pathogen Biology, School of Basic Medicine, Tongji Medical College and State Key Laboratory for Diagnosis and Treatment of Severe Zoonotic Infectious Diseases, Huazhong University of Science and Technology, Wuhan, Hubei, China.
Immune thrombocytopenia (ITP) is an autoimmune disease, characterized by increased bleeding due to a reduced platelet count. The pathogenesis of ITP is very complex and involves autoantibody production and T-cell-mediated immune abnormalities. An imbalance of effector and regulatory CD4 T cells and the breach of tolerance primarily cause ITP, leading to the dysfunctional development of autoreactive Th cells (including Th1, Th2, and Th17 cells) and Tregs.
View Article and Find Full Text PDFGlomerulonephritis After Renal Transplatation in South Asia - Single Center Experience Over 5 Decades.
Indian J Nephrol
July 2024
Department of Nephrology, Christian Medical College Vellore, Ranipet Campus, Ranipet, India.
Background: With significant advances in the understanding of transplant immunology and a reduction in rejection rates, significant improvements in kidney allograft survival have been seen. The problem of recurrent and denovo glomerular diseases after transplantation affecting graft outcomes remains and is poorly characterized. This study aimed to analyze the incidence, characteristics, and outcomes of glomerulonephritis (GN) after kidney transplant in the Indian subcontinent.
View Article and Find Full Text PDF[The combined regimen based on obinutuzumab plus glucocorticoid for 4 cases of relapsed iTTP].
Zhonghua Xue Ye Xue Za Zhi
January 2025
Jiangsu Institute of Hematology, National Clinical Research Center for Hematologic Diseases, NHC Key Laboratory of Thrombosis and Hemostasis, The First Affiliated Hospital of Soochow University, Collaborative Innovation Center of Hematology, Soochow Universtiy, Suzhou 215006, China.
To evaluate the efficacy and safety of obinutuzumab combined with glucocorticoid-based therapy in patients with relapsed immune thrombotic thrombocytopenic purpura (iTTP). This study analyzed the efficacy and adverse reactions of four patients with relapsed iTTP who were treated with a combination of obinutuzumab and glucocorticoids to assess the effectiveness and safety of the treatment. All four patients had a history of multiple relapses and had previously undergone treatment with rituximab and bortezomib.
View Article and Find Full Text PDFReal-world evaluation of a novel automated method for measuring ADAMTS13 activity using the Ceveron® s100 analyzer.
J Thromb Haemost
March 2025
Service d'Hématologie biologique, Hôpital Lariboisière, AP-HP.Nord, Université Paris Cité, Paris, France; INSERM UMR-S 1138, Centre de Recherche des Cordeliers, Université Paris Cité, Sorbonne Université, Paris, France.
Introduction: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by a severe functional deficiency of ADAMTS13. Measuring ADAMTS13 activity is crucial for diagnosing TTP (<10 IU/dL), monitoring treatments, and detecting relapses (<20 IU/dL). The Technofluor® assay allows a rapid ADAMTS13 activity measurement using the CEVERON® s100 analyzer.
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