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The clinical course of the syndrome of essential hyperlipemia is characterized by cutaneous xanthomas, hepatosplenomegaly and recurrent attacks of abdominal pain. The diagnosis is confirmed by the milky appearance of a fasting serum sample. It is interesting to note that 100 years ago Addison and Gull (1) described xanthoma tuberosum as well as xanthoma tendinosum. Seventy-five years were to elapse, however, before Burger and Grutze in 1932 (2) associated the symptoms and objective findings in this disease and described the first case of essential hyperlipemia. Since then more than 50 cases have been reported in the literature; from Sweden, 10 cases were presented by Malmros, Swahn and Truedsson in 1954 (3). Burger and Grutze (2) not only described the syndrome of essential hyperlipemia, but also made important contributions as regards the pathogenesis and therapy of the disease. By alimentary fat load they demonstrated the existence of "Dauerlipoidemi." Treatment with a low-fat diet resulted in disappearance of the cutaneous manifestations and of the hepatosplenomegaly. These findings have since been discussed and confirmed by several writers (4-7). Thannhauser and Stanley (5) fed olive oil labeled with 1131 to patients with essential hyperlipemia and to normal subjects. They found increased concentration and prolonged retention of 13" in the blood of the former group. Havel (8) studied three brothers with essential hyperlipemia who had a prolonged retention of glycerides in the blood stream after a fatty meal. He was able to demonstrate an almost complete deficiency of the heparin-induced lipemia clearing factor. It was suggested that the cause of the hyperlipemia in these cases apparently was related to the metabolism of exogenous glycerides. Schrade, Becker and B6hle (6) have, however, pointed out that completely normal blood lipid levels cannot be obtained in essential hyperlipemia by dietary means. They concluded that there is also a faulty metabolism of endogenous glycerides, a theory which has also been advanced by Schettler, Eggstein and Jobst (9). In view of these observations it seems probable that in essential hyperlipemia there is some defect in the removal of lipids from the blood stream. The following report concerns some studies on the metabolism of the serum glycerides in one case of essential hyperlipemia including the changes in the blood lipid pattern during the course of a viral hepatitis.

the journal of clinical investigation/the journal of clinical investigation

STUDIES ON A CASE OF ESSENTIAL HYPERLIPEMIA. BLOOD LIPIDS, WITH SPECIAL REFERENCE TO THE COMPOSITION AND METABOLISM OF THE SERUM GLYCERIDES BEFORE, DURING AND AFTER THE COURSE OF A VIRAL HEPATITIS