J Korean Soc Pediatr Nephrol > Volume 17(2); 2013 > Article
J Korean Soc Pediatr Nephrol 2013;17(2): 149-153. doi: https://doi.org/10.3339/jkspn.2013.17.2.149
비전형적 혈전성 미세병증 1례
오지영, 박세진, 김기환, 임범진, 정현주, 기정혜, 김기혁, 신재일
1연세대학교 의과대학 소아청소년과
2아주대학교 의과대학 소아청소년과*
3연세대학교 의과대학 임상병리학과†
4국민건강보험 일산병원 임상병리과‡
5국민건강보험 일산병원 소아청소년과§
A Case of Atypical Thrombotic Microangiopathy
Ji Young Oh, Se Jin Park, Ki Hwan Kim, Beom Jin Lim, Hyeon Joo Jeong, Jung Hye Ki, Kee Hyuck Kim, Jae Il Shin
1Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine
2Department of Pediatrics*, Ajou University Hospital, Ajou University School of Medicine
3Department of Pathology†, Yonsei University College of Medicine
4Department of Pathology,‡ National Health Insurance Corporation Ilsan Hospital
5Department of Pediatrics§, National Health Insurance Corporation Ilsan Hospital, Goyang, Korea
Corresponding Author: Jae Il Shin ,Tel: 02-2228-2050, Fax: 02-393-9118, Email: shinji@yuhs.ac
Received: September 20, 2013;  Accepted: October 11, 2013.
This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http:// creativecommons. org/licenses/bync/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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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.
Key words: Fever | Anemia without hemolysis | Thrombocytopenia | Atypical thrombotic microangiopathy (TMA) | Renal biopsy
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