In the human kidney, various patterns of lipid accumulation have been described in patients with genetic defects of lipid metabolism, including familial dysbetalipoproteinemia [34], lecithin-cholesterol acyltransferase deficiency [35], lipoprotein glomerulopathy [36] and alpha-galactosidase A deficiency (Fabry's disease) [37], as well as in patients with acquired conditions such as hypertensive nephrosclerosis [38], focal segmental glomerulosclerosis [39], minimal change disease with massive proteinuria [40] and hepatorenal syndrome [41]. The gene discussed is GLA; the disease is hyperinsulinemic hypoglycemia, familial, 4.