A 26-year-old Chinese male patient with type I glycogen storage diseas
e presented with chronic renal disease, proteinuria, and urolithiasis.
On renal biopsy, focal glomerular sclerosis, increased mesangial matr
ix and cellularity, interstitial fibrosis, tubular atrophy, and promin
ent arteriosclerosis were observed. immunofluorescence microscopy reve
aled Ig A deposits predominantly in the glomerular mesangium. The poss
ible mechanisms of renal involvement in glycogen storage disease are b
riefly discussed.