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KMID : 0811820120160010046
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Journal of Korean Society of Pediatric Nephrology
2012 Volume.16 No. 1 p.46 ~ p.50
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Two Cases of C1q Nephropathy in Sibling
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Kim Su-Young
Kim Seong-Heon
Moon Kyung-Chul
Shin Jae-Il
Jeong Hyeon-Joo
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Abstract
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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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KEYWORD
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C1q nephropathy, Nephrotic syndrome, Steroid resistant
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