ESSENTIAL HYPERLIPEMIA

Abstract

Hyperlipemia of significant degree produces serum lactescence, in contrast to the clear serum of hypercholesteremia and hyperlecithemia. Hyperlipemia after fat ingestion is transient and physiologic; when exaggerated or prolonged, it is pathologic. Essential hyperlipemia, a disease of unknown etiology, is apparently an exaggeration of postprandial hyperlipemia due to a unique metabolic disorder, in contrast to the secondary hyperlipemia in certain other diseases. One or more features of the triad of abdominal pain, hepatosplenomegaly, xanthomata were seen in each of the 21 previously reported cases. The only consistent laboratory abnormality is a total blood lipid increase, predominantly neutral fat, with serum lactescence varying inversely with the resultant decrease in phospholipid/total lipid ratio. Adults differ from children in occasionally showing glycosuria and rarely having a family history of metabolic disease. Therapy is less effective in children, but there is clinical and laboratory improvement in all cases when dietary fat is effectively restricted. The author studied an adult male presenting abdominal pain, hepatomegaly, hyperlipemia and serum lactescence aggravated by a high fat intake and relieved by fat restriction. Sternal marrow biopsy; liver biopsy; studies of renal, hepatic, pancreatic, endocrine and metabolic function were normal. Repair of a paraduodenal hernia did not affect the disease. Injn. of heparin, probably by an emulsifying action, clears fat-induced serum lactescence in vivo; the % clearing being lower in elderly atherosclerotic than normal patients. Spectrophotometric detns. in this young patient showed a low % of heparin-produced clearing after a standard fat meal. This, and the partition of the blood lipid derangement, suggests a predisposition of these patients to atherosclerosis. Therapy of essential hyperlipemia is rigid fat intake restriction; which is effective.