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Improvement of Renal Function by Long-Term Sustained Eculizumab Treatment in a Patient with Paroxysmal Nocturnal Hemoglobinuria
Joint Authors
Yokoyama, Yasuhisa
Ninomiya, Haruhiko
Niiori-Onishi, Akiko
Chiba, Shigeru
Obara, Naoshi
Hasegawa, Yuichi
Sakata-Yanagimoto, Mamiko
Source
Issue
Vol. 2015, Issue 2015 (31 Dec. 2015), pp.1-4, 4 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2015-06-01
Country of Publication
Egypt
No. of Pages
4
Main Subjects
Abstract EN
Chronic kidney disease (CKD) is one of the major manifestations of paroxysmal nocturnal hemoglobinuria (PNH).
CKD in PNH is induced mainly by intravascular hemolysis of PNH-affected red blood cells (RBC) missing the glycosylphosphatidylinositol-anchored proteins with complement-regulatory activities, CD55 and CD59.
CKD develops by heme absorption in the proximal tubules resulting in the interstitial deposition of iron in the kidneys.
We administered eculizumab to a patient with PNH, who was one of 29 patients enrolled in the AEGIS clinical trial, an open-label study of eculizumab in Japan.
The patient was complicated by stage 3 CKD with impaired estimated glomerular filtration rate (eGFR), at grade G3b, and had obvious proteinuria (2-3+, 1-2 g/day).
In a two-year extension to the 12-week AEGIS study, eGFR improved significantly, and the eGFR has since been maintained at grade G2 without proteinuria by sustained eculizumab treatment (>6 years).
Renal function improved and maintained by long-term sustained eculizumab treatment, presumably by clearance of iron from the kidney as well as inhibition of the production of anaphylatoxin C5a, even in advanced stages of CKD, is one of the benefits of eculizumab treatment in PNH.
American Psychological Association (APA)
Ninomiya, Haruhiko& Obara, Naoshi& Niiori-Onishi, Akiko& Yokoyama, Yasuhisa& Sakata-Yanagimoto, Mamiko& Hasegawa, Yuichi…[et al.]. 2015. Improvement of Renal Function by Long-Term Sustained Eculizumab Treatment in a Patient with Paroxysmal Nocturnal Hemoglobinuria. Case Reports in Hematology،Vol. 2015, no. 2015, pp.1-4.
https://search.emarefa.net/detail/BIM-1058625
Modern Language Association (MLA)
Ninomiya, Haruhiko…[et al.]. Improvement of Renal Function by Long-Term Sustained Eculizumab Treatment in a Patient with Paroxysmal Nocturnal Hemoglobinuria. Case Reports in Hematology No. 2015 (2015), pp.1-4.
https://search.emarefa.net/detail/BIM-1058625
American Medical Association (AMA)
Ninomiya, Haruhiko& Obara, Naoshi& Niiori-Onishi, Akiko& Yokoyama, Yasuhisa& Sakata-Yanagimoto, Mamiko& Hasegawa, Yuichi…[et al.]. Improvement of Renal Function by Long-Term Sustained Eculizumab Treatment in a Patient with Paroxysmal Nocturnal Hemoglobinuria. Case Reports in Hematology. 2015. Vol. 2015, no. 2015, pp.1-4.
https://search.emarefa.net/detail/BIM-1058625
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1058625