J Korean Med Sci.  2007 Oct;22(5):928-931. 10.3346/jkms.2007.22.5.928.

Proteinuria in a Boy with Infectious Mononucleosis, C1q Nephropathy, and Dent's Disease

• Lim IS
• Yun KW
• Moon KC
• Cheong HI

• KMID: 1713307
• DOI: http://doi.org/10.3346/jkms.2007.22.5.928

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.

Keyword

C1q Nephropathy; Dent's Disease; CLC-5 Chloride Channel; Epstein-Barr Virus Infections; Proteinuria

MeSH Terms

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

Figure

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  Fig. 1 (A) Light microscopic finding of the renal biopsy shows a glomerulus of normal size and cellularity (periodic acid-Schiff stain; original magnification, ×400). (B) Immunofluorescence microscopic examination reveals dominant mesangial staining of C1q (original magnification, ×200). (C) Electron microscopic examination shows some mesangial electron-dense deposits (original magnification, ×3,500).

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