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http://dx.doi.org/10.3339/jkspn.2012.16.1.46

Two Cases of C1q Nephropathy in Siblings  

Kim, Su-Young (Department of Pediatrics, Pusan National University Children's Hospital)
Kim, Seong-Heon (Department of Pediatrics, Pusan National University Children's Hospital)
Moon, Kyung-Chul (Department of Pathology, SeoulNational University College of Medicine)
Shin, Jae-Il (The Institute of Kidney disease, Pediatrics, Yonsei University Severance Children's Hospital)
Jeong, Hyen-Joo (The Institute of Kidney Disease, Pathology, Yonsei University College of Medicine)
Publication Information
Childhood Kidney Diseases / v.16, no.1, 2012 , pp. 46-50 More about this Journal
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.
Keywords
C1q nephropathy; Nephrotic syndrome; Steroid resistant;
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