J Korean Soc Pediatr Nephrol.
2013 Oct;17(2):149-153.
A Case of Atypical Thrombotic Microangiopathy
- Affiliations
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- 1Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Korea. shinji@yuhs.ac
- 2Department of Pediatrics, Ajou University Hospital, Ajou University School of Medicine, Korea.
- 3Department of Pathology, Yonsei University College of Medicine, Korea.
- 4Department of Pathology, National Health Insurance Corporation Ilsan Hospital, Korea.
- 5Department 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.
Keyword
MeSH Terms
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Acute Kidney Injury
Adolescent
Anemia
Biopsy
Brain
Complement System Proteins
Creatinine
Diagnosis
Edema
Female
Fever
Hemolysis
Hemolytic-Uremic Syndrome
Hospitalization
Humans
Hypertension, Renal
Leukoencephalopathies
Magnetic Resonance Imaging
Nausea
Proteinuria
Purpura, Thrombotic Thrombocytopenic
Resin Cements
Seizures
Thrombocytopenia
Thrombotic Microangiopathies*
Complement System Proteins
Creatinine
Resin Cements
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