Two Cases of C1q Nephropathy in Siblings.
- Author:
Su Young KIM
1
;
Seong Heon KIM
;
Kyung Chul MOON
;
Jae Il SHIN
;
Hyen Joo JEONG
Author Information
1. Department of Pediatrics, Pusan National University Children's Hospital, Yangsan, Korea. suyung@pusan.ac.kr
- Publication Type:Case Report
- Keywords:
C1q nephropathy;
Nephrotic syndrome;
Steroid resistant
- MeSH:
Complement System Proteins;
Cyclosporine;
Hematuria;
Humans;
Hypertension;
Immunoglobulins;
Lupus Erythematosus, Systemic;
Nephrotic Syndrome;
Proteinuria;
Renal Insufficiency;
Siblings
- From:Journal of the Korean Society of Pediatric Nephrology
2012;16(1):46-50
- CountryRepublic of Korea
- Language:Korean
-
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.
