Idiopathic or essential hyperlipemia is an uncommon inherited disorder of fat metabolism in which elevated plasma triglyceride levels lend a characteristic milky appearance to fasting plasma. A wide spectrum of clinical findings in this disorder has been described,1-3 but little is known concerning the mechanism of the biochemical abnormality. Thannhauser4 attributed the high blood lipid levels to delayed plasma clearance of exogenous fat. Schrade et al.5 concluded that the defect is an abnormal metabolism of endogenous glycerides, since completely normal blood lipid levels could not be obtained by dietary restriction of fat. A lipemia-clearing factor was deficient in some cases of essential hyperlipemia6 but not in others.7 Klatskin and Gordon8 noted the coexistence of pancreatitis and the syndrome of essential hyperlipemia, but which was primary is not clear. The problem of pathogenesis is especially important because of the proclivity of patients with this disorder
SIGLER MH, RUBINI ME. Studies with I131 Triolein in Essential Hyperlipemia: Family Study and Discussion of Lipid Transport. Arch Intern Med. 1961;107(6):894–902. doi:10.1001/archinte.1961.03620060094011
Coronavirus Resource Center
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: