Lipemia Retinalis

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     A 45-year-old woman presented with type V phenotype, mixed hyperlipemia and secondary type 2 diabetes mellitus. Her diseases were managed with insulin and oral hypoglycemic agents with combined n–3 fatty acids–orlistat–fibrate–statin lipid-lowering therapy. Despite intensive therapy, the total triglyceride level was typically 4000 mg per deciliter (45 mmol per liter) but ranged up to 26,550 mg per deciliter (300 mmol per liter), the glucose level was 200 to 300 mg per deciliter (11 to 17 mmol per liter), and the glycosylated hemoglobin value was 13 percent. The fibrate-stimulated lipoprotein lipase activity was 0.5 percent of normal. On physical examination, she had eruptive xanthomas mainly on the plantar aspect of her feet. Retinal examination when the triglyceride level was 13,275 mg per deciliter (150 mmol per liter) showed lipemia retinalis (black arrow) and background diabetic retinopathy with blot hemorrhages (white arrow) and hard exudates (arrowhead). Type V phenotype, mixed hyperlipemia is a rare recessive disorder that affects lipoprotein lipase or apolipoprotein C-II and is associated with defective clearance of triglycerides, eruptive xanthomas, recurrent pancreatitis, and secondary diabetes. Intensive insulin therapy is used to improve hyperglycemia and total triglyceride levels.

    Jesse Kumar, M.R.C.P.

    Anthony S. Wierzbicki, F.A.H.A.

    St. Thomas' Hospital

    London SE1 7EH, United Kingdom

    http://www.100md.com/html/DirDu/2007/01/01/32/57/40.htm