Abstract
We report a 4-years-old girl with Alport syndrome as an acute onset of acute nephritic syndrome. She was referred to our hospital for further examination of edema, severe proteinuria and macrohematuria, 7 days after onset. These symptoms except for microhematuria were improved gradually by bed rest, restriction of salt and water intake, anticoagulant therapy. On renal biopsy, there was no deposit of IgA on immunofluorescence microscopic examination. Light microscopy with periodic acid-Schiff (PAS) stain showed mild mesangial proliferation. On electron microscopic examination, glomerular basement membrane appeared segmental thickness and thinness. Glomerular basement membrane revealed no staining for α 5 chain of collagen IV. She was diagnosed as Alport syndrome.
Acute nephritic syndrome is characterized clinically by sudden-onset proteinuria and/or hematuria. Most cases of acute nephritic syndrome are caused by poststreptococcal acute glomerulonephritis and acute onset/exacerbation of chronic glomerulonephritis. Based on our experience, it is important that Alport syndrome can be one cause of acute nephritic syndrome.
