J Korean Soc Pediatr Nephrol > Volume 16(1); 2012 > Article
J Korean Soc Pediatr Nephrol 2012;16(1): 46-50. doi: https://doi.org/10.3339/jkspn.2012.16.1.46
남매에서 발생한 C1q 신증 2례
김수영, 김성헌, 문경철, 신재일, 정현주
1부산대학교 어린이병원 소아청소년과
2부산대학교 어린이병원 소아청소년과
3서울대학교 의과대학 병리학교실
4연세대학교 세브란스 어린이 병원 소아과
5연세대학교 의과대학 신장연구소 병리학교실
Two Cases of C1q Nephropathy in Siblings
Su-Young Kim, Seong-Heon Kim, Kyung-Chul Moon, Jae-Il Shin, Hyen-Joo Jeong
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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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.
Key words: C1q nephropathy | Nephrotic syndrome | Steroid resistant

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