J Korean Soc Pediatr Nephrol.
2012 Apr;16(1):46-50.
Two Cases of C1q Nephropathy in Siblings
- Affiliations
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- 1Department of Pediatrics, Pusan National University Children's Hospital, Yangsan, Korea. suyung@pusan.ac.kr
- 2Department of Pathology, Seoul National University College of Medicine, Seoul, Korea.
- 3The Institute of Kidney disease, Pediatrics, Yonsei University Severance Children's Hospital, Seoul, Korea.
- 4The Institute of Kidney Disease, Pathology, Yonsei University College of Medicine, Seoul, Korea.
Abstract
- C1q nephropathy is a distinct clinicopathologic entity, characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence for systemic lupus erythematosus. Clinically it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microscopic hematuria, gross hematuria, hypertension, or renal insufficiency. So far there is only one report about a familial case of C1q nephropathy (in two sisters). We present two cases of familial C1q nephropathy with nephrotic syndrome which was steroid resistant, but partially remitted with cyclosporine.
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