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비전형적 혈전성 미세병증 1례

Other Titles
 A Case of Atypical Thrombotic Microangiopathy 
Authors
 Ji Young Oh  ;  Se Jin Park  ;  Ki Hwan Kim  ;  Beom Jin Lim  ;  Hyeon Joo Jeong  ;  Jung Hye Ki  ;  Kee Hyuck Kim  ;  Jae Il Shin 
Citation
 Journal of the Korean Society of Pediatric Nephrology (대한소아신장학회지), Vol.17(2) : 149-153, 2013 
Journal Title
 Journal of the Korean Society of Pediatric Nephrology (대한소아신장학회지) 
ISSN
 1226-5292 
Issue Date
2013
Keywords
Fever ; Anemia without hemolysis ; Thrombocytopenia ; Atypical thrombotic microangiopathy (TMA) ; Renal biopsy
Abstract
We report the case of a 14-year-old girl, diagnosed with atypical thrombotic microangiopathy (TMA). The patient presented with persistent fever, nausea, and newly developed peripheral edema. Her laboratory findings indicated chronic anemia with no evidence of hemolysis, thrombocytopenia, or elevated serum creatinine level. A few days after hospitalization, acute renal failure and fever worsened, and proteinuria developed. On day 40 of hospitalization, she experienced a generalized tonic seizure for 5 min, accompanied by renal hypertension. Brain magnetic resonance imaging revealed posterior reversible leukoencephalopathy syndrome. After steroid pulse therapy, a renal biopsy was performed because of delayed recovery from thrombocytopenia. The biopsy findings showed features of thrombotic microangiopathic hemolysis with fibrinoid change restricted. Current diagnostic criteria for TMA have focused on thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, and diagnosis is based on the clinical presentation and etiology, with the consequence that idiopathic and atypical forms of TMA can be overlooked. Developing effective tools to diagnose TMA, such as studying levels of ADAMTS13 or testing for abnormalities in the complement system, will be the first step to improving patient outcomes.
Files in This Item:
T201305641.pdf Download
DOI
10.3339/jkspn.2013.17.2.149
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Ki Hwan(김기환)
Shin, Jae Il(신재일) ORCID logo https://orcid.org/0000-0003-2326-1820
Oh, Ji Young(오지영) ORCID logo https://orcid.org/0000-0003-3552-8465
Lim, Beom Jin(임범진) ORCID logo https://orcid.org/0000-0003-2856-0133
Jeong, Hyeon Joo(정현주) ORCID logo https://orcid.org/0000-0002-9695-1227
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/89142
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