Abstract
Lipoprotein lipase activity in lipomatous tissue, post-heparin lipoprotein lipase activity in plasma, and the composition and concentration of serum lipoproteins were studied in 15 patients with Multiple Symmetric Lipomatosis (MSL). Extremely elevated lipoprotein lipase activity in adipose tissue was found in MSL patients. Total and hepatic post-heparin plasma lipolytic activity was normal, while a moderate but statistically significant increase of extrahepatic lipolytic activity was present. An abnormal composition of serum lipoproteins, characterized by a significant increase in high density lipoproteins, namely HDL2 subfraction, and apoprotein A-I, was demonstrated. A concomitant decrease in and abnormal composition of low density lipoproteins were found. This lipoprotein pattern is consistent with a newly recognized type of hyperalphalipoproteinemia. Significant correlations were found between serum HDL2 cholesterol values and lipoprotein lipase activity in adipose tissue (as well as between serum VLDL-triglyceride and HDL2 cholesterol values). These observations confirm the role of adipose tissue lipoprotein lipase in triglyceride-rich lipoprotein catabolism. The elevated levels of lipoprotein lipase activity in adipose tissue, in addition to a previously demonstrated decrease in adrenergic-stimulated lipid mobilization, could account for both the abnormal fat accumulation in lipomatous fat cell and for hyperalphalipoproteinemia in MSL patients. The occurrence of MSL in two brothers suggests an inherited enzymatic defect, indicating MSL as a "triglyceride storage disease in adipose tissue".
Highlights
Lipoprotein lipaseactivityin lipomatoustissue,postheparin lipoprotein lipase activity in plasma, and the composition and concentration of serum lipoproteins were studied in 15 patients with Multiple Symmetric Lipomatosis (MSL)
T h e presence of slowly growing, symmetrically disposed fat masses at the neck, the shoulders, the deltoid region, the trunk, and groin characterizes the outward feature of patients with multiple symmetric lipomatosis (MSL) (Fig. 1)
T h e aim of this study is to evaluate possible alterations in lipoprotein lipase (LPL) activities and in circulating lipoprotein composition in a group of MSL patients large enough to provide definitive information
Summary
Fifteen male patients from northeastern Italy aged 34-65 years and affected by MSL were studied during a short hospital stay. Alcohol intake, obtained by a 7-day recall, ranged between 60 and 220 ml per day. Body fat mass was calculated according to the Edwards and White formula [13], using height and body weight as parameters. Address reprint requests to: Dr Giuliano Enzi, Department of Internal Medicine, Policlinico-Via Giustiniani, 2, 35 100 Padova, Italy. For the study, which was approved by the ethical committee of the Medical Faculty
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