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September 1967

Lipids in Blood Plasma and Erythrocytes in Juvenile Amaurotic Idiocy: Cholestyramine Therapy

Author Affiliations

Amsterdam and Leiden, the Netherlands
From the Pediatric Clinic of the University of Amsterdam and the Department of Medical Chemistry, State-University of Leiden, the Netherlands.

Arch Neurol. 1967;17(3):225-229. doi:10.1001/archneur.1967.00470270003001

IN THE study of disturbances in lipid metabolism—with or without cerebral symptoms—lipids of erythrocytes and plasma or serum have also been implicated recently.1-14 In generalized disorders of lipid metabolism, even when manifest by localization in certain tissues, abnormalities of plasma and erythrocytes might be of diagnostic significance or important for genetic studies.

Cholestyramine resin (Questran) is a basic anion-exchange resin with a special affinity for bile acids. After oral administration of cholestyramine resin, the bile acids are sequestered in the gut, thus preventing their reabsorption and promoting excretion in the feces. In this way the oxidation conversion of cholesterol to bile acids should be intensified, followed by a decrease of serum cholesterol (and possibly in other tissues). There are indications that the absorption of dietary cholesterol from the intestine may also be prevented by cholestyramine resin. Administration of cholestyramine resin lowers serum cholesterol in such conditions as intrahepatic