1Department of Pediatrics, Pusan National University Children's Hospital 2Department of Pediatrics, Pusan National University Children's Hospital 3Department of Pathology, SeoulNational University College of Medicine 4The Institute of Kidney disease, Pediatrics, Yonsei University Severance Children's Hospital 5The Institute of Kidney Disease, Pathology, Yonsei University College of Medicine
Received: July 11, 2011; Accepted: August 31, 2011.
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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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