SUBCLINICAL FABRYS-DISEASE OCCURRING IN THE CONTEXT OF IGA NEPHROPATHY

A 28-year-old male patient with both IgA nephropathy and an unusual ca se of Fabry's disease has been followed for 10 years. Diagnosis of bot h these diseases was made by histological examination of renal biopsy tissues and the enzyme activities of alpha-galactosidase A. Serial bio psies revealed the hithertofore unrecognized process of glomerular gly colipid accumulation peculiar to Fabry's disease at the initial stages of the disease. Physical examinations and routine laboratory analyses failed to show significant signs of Fabry's disease throughout the 10 -year period, While alpha-galactosidase A activity is markedly decreas ed in the plasma of this patient as in classical Fabry hemizygotes, th e activity in leukocytes and culture fibroblasts showed a considerable residual activity. Fabry's disease associated with IgA nephropathy ap parently is extremely rare, and the present subclinical case is unique in that the early stages of substrate accumulation are demonstrable.