Dyslipoproteinemia and an inhibitor of lipolytic enzymes in Weber-Christian disease.

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 microliters of post-heparin plasma than with more than 30 microliters, 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 less than d less than 1.040). These results are consistent with the idea that dyslipoproteinemia in this patient was partially due to dysfunction of the catabolic system caused with an inhibitor of lipolytic enzymes.