Results 71 to 80 of about 12,992 (201)

Monoclonal IgG4/2κ Deposition Following Eculizumab Therapy for Recurrent Atypical Hemolytic Uremic Syndrome in Kidney Transplantation

Kidney Medicine, 2019
Eculizumab is an emerging therapy for atypical hemolytic uremic syndrome (aHUS). Early identification and treatment of recurrent aHUS after kidney transplantation requires a high clinical suspicion but results in improved graft function and patient ...
Priyamvada Singh, Hui Chen, Craig E. Gordon, Sandeep Ghai, J. Mark Sloan, Karen Quillen, Sara Moradi, Vipul Chitalia, Amitabh Gautam, Joel Henderson, Jean M. Francis   +10 more
doaj   +1 more source

Management of Refractory Thymoma‐Associated Myasthenia Gravis With Sequential FcRn Antagonist and Complement Inhibition Combined With Resection of Pleural Dissemination

Neurology and Clinical Neuroscience, EarlyView.
ABSTRACT Myasthenia gravis (MG) associated with disseminated thymoma is often refractory to conventional treatments due to continuous autoantibody production. The optimal treatment strategy, particularly the combination of complement component 5 (C5) inhibitor, neonatal Fc receptor (FcRn) antagonist, and surgical resection in thymoma‐associated ...
Hiroyuki Takenaka, Madoka Iida, Yuki Fukami, Masahisa Katsuno   +3 more
wiley   +1 more source

Eculizumab Dosing Regimen in Atypical HUS: Possibilities for Individualized Treatment [PDF]

, 2017
Contains fulltext : 177003.pdf (Publisher’s version ) (Open Access) Contains fulltext : 177003pos.pdf (Author’s version postprint ) (Open Access)Recent studies indicate that ...
Wijnsma, K.L., Velden, T.J. van der, Goertz, J.H.C., Roeleveld, N., Heuvel, L.P. van den, Sweep, C.G.J., Kar, N.C. van de, Bruggemann, R.J.M., Volokhina, E.B., Wetzels, J., Molen, R.G. van der   +10 more
core   +3 more sources

Eculizumab in Pediatric Dense Deposit Disease

, 2015
Dense deposit disease (DDD), a subtype of C3 glomerulopathy, is a rare disease affecting mostly children. Treatment options are limited. Debate exists whether eculizumab, a monoclonal antibody against complement factor C5, is effective in DDD.
Bouts, Antonia H. M., Hoppe, Bernd, Roelofs, Joris J. T. H., Schriemer, Pietrik J., Florquin, Sandrine, Amann, Kerstin, Groothoff, Jaap W., Garrelfs, Mark R., van den Heuvel, L. P., Oosterveld, Michiel J. S., Davin, Jean-Claude   +10 more
core   +1 more source

Deficiency of high‐molecular‐weight von Willebrand factor mitigates thrombo‐complement injury in an LPS‐induced TMA‐like mouse model

VIEW, EarlyView.
Reduction of high‐molecular‐weight von Willebrand factor disrupts the platelet–complement amplification loop, attenuating microvascular thrombosis, complement deposition, endothelial injury, and organ damage in complement‐mediated thrombotic microangiopathy.
Yang Li, Chihao Zhang, Xiaoqian Xu, Sicong Wang, Xiaofan Hu, Junjie Yao, Xi Wu, Difeng Zhu, Qian Liang, Yeling Lu, Yu Liu, Guanqun Xu, Jiakuan Zhou, Qiulan Ding, Wenman Wu, Ya Li, Jing Dai, Xuefeng Wang   +17 more
wiley   +1 more source

Patients receiving Eculizumab (Soliris\uae) at high risk for invasive meningococcal disease despite vaccination [PDF]

July 7, 2017, 11:00 ET (11:00 AM ET)CDCHAN-00404Summary: Eculizumab (Soliris\uae) recipients have a 1,000 to 2,000-fold greater risk of invasive meningococcal disease compared to the general U.S. population.

core  

Inborn errors of immunity in children with neuroinflammation

Developmental Medicine &Child Neurology, EarlyView.
Abstract Inborn errors of immunity (IEIs), an expanding group of monogenic disorders with diverse clinical manifestations, are increasingly recognized to include neuroinflammatory disease. Examples of diseases included under this umbrella are Aicardi–Goutières syndrome, deficiency of adenosine deaminase 2, familial haemophagocytic lymphohistiocytosis ...
Eppie M Yiu, Dilan Dissanayake, Jessie Cunningham, Helen Branson, Ori Scott, E Ann Yeh   +5 more
wiley   +1 more source

Clonal Dynamics of GPI‐Deficient Cells in Patients With Paroxysmal Nocturnal Hemoglobinuria (PNH): A Retrospective Follow‐Up Analysis

European Journal of Haematology, EarlyView.
ABSTRACT This retrospective, single‐center study aimed to characterize clonal dynamics of GPI‐deficient cells in patients with paroxysmal nocturnal hemoglobinuria (PNH) or PNH/aplastic anemia (AA) syndrome using multiparameter flow cytometry including FLAER.
Sandra M. Frey, Friederike Poppenborg, Ute Schmücker, Nicole Preising, H. Christian Reinhardt, Alexander Röth, Ferras Alashkar   +6 more
wiley   +1 more source

Behandling med eculizumab vid katastrofalt antifosfolipidsyndrom

, 2018
Bakgrund: Eculizumab (Solirisâ) är en monoklonal antikropp som är riktad mot C5 i komplementsystemet. Bindning av eculizumab till C5 förhindrar proteinets klyvning och därmed också aktivering.
Cronin, Jennifer
core   +2 more sources

Expert Consensus on the Diagnosis and Monitoring of Paroxysmal Nocturnal Hemoglobinuria (PNH): An Algorithmic Approach in an Era of New Treatments

European Journal of Haematology, EarlyView.
ABSTRACT Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder caused by a defect of glycosylphosphatidyl‐anchored proteins, leading to an uncontrolled complement‐mediated hemolysis. The advent of complement inhibitors in clinical practice radically changed patients' outcomes and survival.
Bruno Fattizzo, Monia Marchetti, Elisa Cannizzo, Arianna Gatti, Massimo Geuna, Anna Paola Iori, Maddalena Raia, Simona Sica, Francesco Buccisano   +8 more
wiley   +1 more source