Renal Lesion of Type la Glycogen Storage Disease: The Glomerular Size and Renal Localization of Apolipoprotein

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Abstract
In order to investigate the glomerular size and renal localization of apolipoprotein in type la 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 × 103 μm2, indicating enlargement of the glomeruli. Immunohistochemical staining of the specimens for apolipoprotein showed localization of apolipoprotein AI on the inner side of the glomerular capillary wall, and in proximal tubular epithelial cells. In one patient with a history of several episodes of hypoglycemia, treatment with corn starch improved the carbohydrate and lipid metabolic profile and reduced the daily urinary protein excretion from 2.23 to 0.5 g. These results suggest that focal sclerotic glomerular lesions associated with type la glycogen storage disease may be related to disorders of carbohydrate and lipid metabolism.

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