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著者: Peter Hillmen, Modupe Elebute, Richard Kelly, Alvaro Urbano-Ispizua, Anita Hill, Russell P Rother, Gus Khursigara, Chieh-Lin Fu, Mitsuhiro Omine, Paul Browne, Wendell Rosse
雑誌名: Am J Hematol. 2010 Aug;85(8):553-9. doi: 10.1002/ajh.21757.
Abstract/Text
Paroxysmal nocturnal hemoglobinuria (PNH) is a debilitating and life-threatening disease in which lysis of PNH red blood cells frequently manifests with chronic hemolysis, anemia, and thrombosis. Renal damage in PNH is associated with chronic hemosiderosis and/or microvascular thrombosis. We determined the incidence of renal dysfunction or damage, defined by stages of chronic kidney disease (CKD), in a large cohort of PNH patients and evaluated the safety and efficacy of the complement inhibitor eculizumab in altering its progression. Renal dysfunction or damage was observed in 65% of the study population at baseline with 21% of patients with later stage CKD or kidney failure (glomerular filtration rate [GFR]
(c) 2010 Wiley-Liss, Inc.
PMID 20658586 Am J Hematol. 2010 Aug;85(8):553-9. doi: 10.1002/ajh.21757.
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