RENAL LESION OF TYPE IA GLYCOGEN-STORAGE-DISEASE - THE GLOMERULAR SIZE AND RENAL LOCALIZATION OF APOLIPOPROTEIN

In order to investigate the glomerular size and renal localization of apolipoprotein in type Ia glycogen storage disease, a renal biopsy was performed in two proteinuric patients. Histopathological examination of the biopsy specimens revealed focal sclerotic glomerular sclerosis in both patients. The mean glomerular area was 21.6 +/- 11.6 x 10(3) m u m(2), indicating enlargement of the glomeruli. Immunohistochemical s taining of the specimens for apolipoprotein showed localization of apo lipoprotein AI on the inner side of the glomerular capillary wall, and in proximal tubular epithelial cells. In one patient with a history o f several episodes of hypoglycemia, treatment with corn starch improve d the carbohydrate and lipid metabolic profile and reduced the daily u rinary protein excretion from 2.23 to 0.5 g. These results suggest tha t focal sclerotic glomerular lesions associated with type Ia glycogen storage disease may be related to disorders of carbohydrate and lipid metabolism.