Proteinuria in a Boy with Infectious Mononucleosis, C1q Nephropathy, and Dent's Disease.
10.3346/jkms.2007.22.5.928
- Author:
In Seok LIM
1
;
Ki Wook YUN
;
Kyung Chul MOON
;
Hae Il CHEONG
Author Information
1. Department of Pediatrics, Chungang University Yongsan Hospital, Seoul, Korea. inseok@cau.ac.kr
- Publication Type:Case Report ; Research Support, Non-U.S. Gov't
- Keywords:
C1q Nephropathy;
Dent's Disease;
CLC-5 Chloride Channel;
Epstein-Barr Virus Infections;
Proteinuria
- MeSH:
Biopsy;
Child, Preschool;
Epstein-Barr Virus Infections/metabolism;
Glomerulonephritis/pathology;
Humans;
In Situ Hybridization;
Infectious Mononucleosis/*complications/*diagnosis;
Kidney Diseases/*complications/*diagnosis;
Kidney Tubules/*pathology;
Male;
Membrane Glycoproteins/*chemistry;
Mutation;
Nephrosis;
Proteinuria/*complications/*diagnosis;
Receptors, Complement/*chemistry;
Treatment Outcome
- From:Journal of Korean Medical Science
2007;22(5):928-931
- CountryRepublic of Korea
- Language:English
-
Abstract:
C1q nephropathy is a proliferative glomerulopathy with extensive mesangial deposition of C1q. A three-year old boy presented with a nephrotic-range proteinuria during an acute phase of Epstein-Barr virus (EBV) infection, and he had a family history of Dent's disease. The renal biopsy findings were compatible with C1q nephropathy. However, EBV in situ hybridization was negative. The CLCN5 gene analysis revealed an R637X hemizygous mutation, which was the same as that detected in his maternal cousin, the proband of the family. The causal relationship between EBV infection and C1q nephropathy remains to be determined. Moreover, the effects of underlying Dent's disease in the process of C1q nephropathy has to be considered.
