Journal Browser Advanced Search Help
Journal Browser Advanced search HELP
J Korean Soc Pediatr Nephrol. 2013 Oct;17(2):149-153. English. Case Report.
Oh JY , Park SJ , Kim KH , Lim BJ , Jeong HJ , Ki JH , Kim KH , Shin JI .
Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Korea. shinji@yuhs.ac
Department of Pediatrics, Ajou University Hospital, Ajou University School of Medicine, Korea.
Department of Pathology, Yonsei University College of Medicine, Korea.
Department of Pathology, National Health Insurance Corporation Ilsan Hospital, Korea.
Department of Pediatrics, National Health Insurance Corporation Ilsan Hospital, Goyang, Korea.
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.

Copyright © 2019. Korean Association of Medical Journal Editors.