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10.12688/f1000research.7288.1 http://scihub22266oqcxt.onion/10.12688/f1000research.7288.1 C4765720!4765720!26962442     free     free     free Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       F1000Res 2016 ; 5 (ä): ä Nephropedia Template TP {{tp|p=26962442|t=2016. Recent advances in the pathogenesis and treatment of paroxysmal nocturnal hemoglobinuria |pdf=|usr=}}{{26962442}} gab.com Text Recent advances in the pathogenesis and treatment of paroxysmal nocturnal hemoglobinuria JO: F1000Res http://www.kidney.de/pget.php?&tw=1&pmid=26962442 #FOAvip Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare disease that has been investigated for over one century and has revealed unique aspects of the pathogenesis and pathophysiology of a hemolytic anemia. PNH results from expansion of a clone of hematopoietic cells that, as a consequence of an inactivating mutation of the X-linked gene PIG-A, are deficient in glycosylphosphatidylinositol (GPI)-linked proteins: since these include the surface membrane complement-regulatory proteins CD55 and CD59, the red cells arising from this clone are exquisitely sensitive to lysis by activated complement. Until a decade ago, the treatment options for PNH were either supportive treatment ? often including blood transfusion, anti-thrombosis prophylaxis, and sometimes thrombolytic therapy ? or allogeneic bone marrow transplantation. Since 2007, PNH has received renewed and much wider attention because a new form of treatment has become available, namely complement blockade through the anti-C5 monoclonal antibody eculizumab. This brief review focuses on two specific aspects of PNH: (1) response to eculizumab, variability of response, and how this new agent has impacted favorably on the outlook and on the quality of life of patients; and (2) with respect to pathogenesis, new evidence supports the notion that expansion of the PNH clone results from T-cell-mediated auto-immune damage to hematopoietic stem cells, with the GPI molecule as target. Indeed, GPI-specific CD8+ T cells ? which have been identified in PNH patients ? would spare selectively GPI-negative stem cells, thus enabling them to re-populate the marrow of a patient who would otherwise have aplastic anemia. Twit Text FOAVip Recent advances in the pathogenesis and treatment of paroxysmal nocturnal hemoglobinuria ????F1000Res http://www.kidney.de/pget.php?&tw=1&pmid=26962442 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=26962442 #FOAvip English Wikipedia <ref>{{Cite pmid|26962442}}</ref> Recent advances in the pathogenesis and treatment of paroxysmal nocturnal hemoglobinuria #MMPMID26962442 Luzzatto L F1000Res 2016[]; 5 (ä): ä PMID26962442show ga Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare disease that has been investigated for over one century and has revealed unique aspects of the pathogenesis and pathophysiology of a hemolytic anemia. PNH results from expansion of a clone of hematopoietic cells that, as a consequence of an inactivating mutation of the X-linked gene PIG-A, are deficient in glycosylphosphatidylinositol (GPI)-linked proteins: since these include the surface membrane complement-regulatory proteins CD55 and CD59, the red cells arising from this clone are exquisitely sensitive to lysis by activated complement. Until a decade ago, the treatment options for PNH were either supportive treatment ? often including blood transfusion, anti-thrombosis prophylaxis, and sometimes thrombolytic therapy ? or allogeneic bone marrow transplantation. Since 2007, PNH has received renewed and much wider attention because a new form of treatment has become available, namely complement blockade through the anti-C5 monoclonal antibody eculizumab. This brief review focuses on two specific aspects of PNH: (1) response to eculizumab, variability of response, and how this new agent has impacted favorably on the outlook and on the quality of life of patients; and (2) with respect to pathogenesis, new evidence supports the notion that expansion of the PNH clone results from T-cell-mediated auto-immune damage to hematopoietic stem cells, with the GPI molecule as target. Indeed, GPI-specific CD8+ T cells ? which have been identified in PNH patients ? would spare selectively GPI-negative stem cells, thus enabling them to re-populate the marrow of a patient who would otherwise have aplastic anemia. äRecent advances in the pathogenesis and treatment of paroxysmal noctur(epmc).pdf DeepDyve Pubget Overpricing