A case of atypical hemolytic uremic syndrome with a transient decrease in complement factor H |
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Authors: | Hyewon Hahn Eun Young Um Young Seo Park Hae Il Cheong |
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Affiliation: | (1) Department of Pediatrics, Eulji University School of Medicine, Daejeon, Korea;(2) Department of Pediatrics, Seoul National University College of Medicine, Clinical Research Institute, Seoul, Korea;(3) Department of Pediatrics, University of Ulsan College of Medicine, Ulsan, Korea;(4) Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea |
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Abstract: | We report a case of sporadic atypical hemolytic uremic syndrome (HUS) with a transient decrease in complement factor H. Referred for hemolysis and azotemia without diarrhea prodrome, this 31-month-old boy showed a decreased complement 3 (C3) and complement factor H (FH) level. However, the factor H gene (HF1) mutation was missing. After the hemolysis was controlled with plasma infusion, the C3 and FH levels recovered. The patients renal function fully recovered and remained normal, and there was no recurrence of the HUS. |
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Keywords: | Complement 3 Complement factor H Hemolytic-uremic syndrome |
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