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A Successfully Treated Case of Recurrent Focal Segmental Glomerulosclerosis (FSGS) with Plasmapheresis and High dose Methylprednisolone Pulse Therapy

Authors
 Sun Mi Her  ;  Keum Hwa Lee  ;  Jae Il Shin 
Citation
 Childhood Kidney Diseases, Vol.21(2) : 165-168, 2017 
Journal Title
 Childhood Kidney Diseases 
ISSN
 2384-0250 
Issue Date
2017
Keywords
Focal segmental glomerulosclerosis ; End stage renal disease ; Soluble urokinase-type plasminogen activator receptor ; Anti-CD40 autoantibody
Abstract
Focal segmental glomerulosclerosis (FSGS) in children, which is a kind of nephrotic syndrome showing steroid resistance, usually progresses to a substantial number of end stage renal disease (ESRD). Although the pathogenesis of primary FSGS is unclear, several recent studies have reported that FSGS is associated with circulating immune factors such as soluble urokinase-type plasminogen activator receptor (suPAR) or anti-CD40 autoantibody. We report a successfully treated case of a 19-year-old female patient who experienced a recurrence of primary FSGS. After the diagnosis of FSGS, the patient progressed to ESRD and received a kidney transplantation (KT). Three days later, recurrence was suspected through proteinuria and hypoalbuminemia. She has been performed plasmapheresis and high dose methylprednisolone pulse therapy and shown remission status without increasing proteinuria for four years after KT. In conclusion, strong immunosuppressive therapy may be helpful for a good prognosis of recurrent FSGS, suppressing several immunologic circulating factors related disease pathogenesis.
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DOI
10.3339/jkspn.2017.21.2.165
Appears in Collections:
1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실)
Yonsei Authors
신재일(Shin, Jae Il) ORCID logo https://orcid.org/0000-0003-2326-1820
이금화(Lee, Keum Hwa) ORCID logo https://orcid.org/0000-0002-1511-9587
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URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/161302
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