Abstract:

ABSTRACT: A 16-year-old female, who presented with nephrotic syndrome and relieved with prednisone therapy, was reported. The renal biopsy was performed. The light microscope showed mild mesangial proliferation, immunofluorescence staining showed typical high-intensity staining for IgG、IgA and C1q, and electron microscope showed mesangial electron-dense deposits and extensive foot processes effacement of podocyte. Two years later, her nephritic syndrome relapsed, and the damage of multiple organ systems was developed. The analysis of her mitochondrial DNA revealed an G-to-A mutation of the tRNA leu(UUR) gene at the 8969 position. The patient was finally diagnosed as IgA nephropathy associated with mitochondrial cytopathy.

Key words: Key words , mitochondrial cytopathy , renal biopsy , IgA nephropathy