LIPOPROTEIN GLOMERULOPATHY - FIRST CASE IN A WHITE EUROPEAN

Since 1988, 11 cases of a new entity, 'Lipoprotein glomerulopathy' (LG ), were described in Japan. Some of these reports suggested that this glomeruler lipid storage is due to excess apo E associated with hetero zygous E2/3 apo E isoform. We report the first case of LG in a white E uropean with no such lipid abnormalities. Proteinuria was discovered i n 1967 when he was 42. Blood pressure and renal function were normal. Family history was negative. Renal biopsy disclosed lesions which were only understood at the time of the Japanese publications. They were c omposed of endocapillary glomerular deposits. Staining for lipids disc losed capillary loop obstruction with lipid droplets. Electron microsc opy showed confluent droplets of various sizes obstructing capillary l oops. Proteinuria progressively increased. In 1974 repeat renal biopsy showed the same lipid deposits, now associated with focal-segmental g lomerulosclerosis (FSGS). Several serum lipoprotein and apolipoprotein studies ruled out any specific lipid derangement. This suggested a lo cal glomerular disorder, presumably affecting the glomerular endocapil lary disposal of lipids. A third biopsy showed progressive glomerular destruction by FSGS with persistence of the lipid droplets. Renal insu fficiency progressed and haemodialysis was started in 1992. This obser vation suggests that LG is a local glomerular, not a general lipid dis order and indicates that this disease is not restricted to Asian patie nts.