Two Cases of C1q Nephropathy in Siblings

Kim, Su-Young; Kim, Seong-Heon; Moon, Kyung-Chul; Shin, Jae-Il; Jeong, Hyen-Joo

doi:2012.16.1.46

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.

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.

Key words: C1q nephropathy | Nephrotic syndrome | Steroid resistant