Dyslipoproteinemia and an Inhibitor of Lipolytic Enzymes in Weber-Christian Disease

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Hyperlipidemia of initially Type V and finally of Type III was observed in a patient with Weber-Christian disease. The lipoprotein lipase (LpL) and hepatic triglyceride lipase (HTGL) activities of the post-heparin plasma were low, but detectable. Both lipase activities were higher when assayed with 20 μl of post-heparin plasma than with more than 30 μl indicating the presence of some inhibitory factor in the plasma. This plasma inhibited purified LpL and HTGL from human post-heparin plasma. Zonal ultracentrifugation studies showed that the inhibitor of hepatic lipase was associated with the middle fraction of intermediate lipoprotein and low density lipoproteins (1.020<d<1.040). These results are consistent with the idea that dyslipoprotenemia in this patient was partially due to dysfunction of the catabolic system caused with an inhibitor of lipolytic enzymes.