Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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C3 Glomerulonephritis associated with Anti-complement Factor H Autoantibodies in an Adolescent Male: A Case Report

  • Hyun, HyeSun (Department of Pediatrics, St. Vincent's Hospital, The Catholic University of Korea) ;
  • Kang, Hee Gyung (Department of Pediatrics, Seoul National University Children's Hospital) ;
  • Cho, UiJu (Department of Pathology, St. Vincent's Hospital, The Catholic University of Korea) ;
  • Ha, Il-Soo (Department of Pediatrics, Seoul National University Children's Hospital) ;
  • Cheong, Hae Il (Department of Pediatrics, Seoul National University Children's Hospital)
  • Received : 2020.12.31
  • Accepted : 2021.03.19
  • Published : 2021.06.30
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Abstract

C3 glomerulonephritis (C3GN), a rare condition associated with dysregulation of the alternative pathway of the complement system, is histopathologically characterized by isolated or dominant C3 deposition in the renal glomeruli. We report a case of C3GN associated with anti-complement factor H (CFH) autoantibodies and CHF-related protein deficiency in an adolescent male. A 16-year-old adolescent male was admitted to a hospital with a 1-month history of generalized edema prior to presentation. Persistent microscopic hematuria and low serum C3 levels were incidentally detected at 7 and 10 years of age, respectively. Laboratory test results revealed hypoalbuminemia, nephrotic-range proteinuria, microscopic hematuria, and normal serum creatinine levels. The serum C3 and C4 levels were 17 mg/dL (normal 80-150 mg/dL) and 22 mg/mL (17-40 mg/mL), respectively. Renal biopsy showed typical features of C3GN. Further investigations revealed positive results on plasma anti-CFH autoantibody testing and a homozygous deletion of CFHR1 and CFHR3, which encode CFH-related proteins 1 and 3, respectively. Proteinuria persisted despite treatment with intravenous methylprednisolone, mycophenolate mofetil, and angiotensin-receptor blocker; however, his renal function remained stable. In conclusion, anti-CFH autoantibodies serve as important contributors to C3GN. This is the first case report that describes C3GN in an adolescent Korean male with anti-CFH autoantibodies and homozygous CFHR1 and CFHR3 deletion.

Keywords

Acknowledgement

This research was supported by a grant of the Korea Health Technology Research & Development Project through the Korea Health Industry Development Institute, funded by the Ministry of Health & Welfare, Republic of Korea (grant number: HI18C0013).

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