Abstract:

A 33 yearold female presented with mild to moderate proteinuria, mild hematuria, normotension, and normal renal function for 5 months. She had no extrarenal symptom or family history of Fabry disease. A renal biopsy showed mild mesangial expansion and strikingly enlarged and vacuolated podocytes. On immunofluorescence microscopy, IgA and C3 deposits were detected in mesangial areas. Semithin section stained with toluidin blue showed characteristic “blue inclusions” in podocytes. Myeloid bodies and zebra bodies were detected mainly in podocytes characteristic of Fabry disease, and dense deposits were detected in mesangial area by electron microscopy. The final diagnosis was coexistence of Fabry disease and IgA nephropathy．