Howard R. Sloan, Donald S. Fredrickson
J Clin Invest.
1972;
51(7):1923–1926
doi:10.1172/JCI106997
This article Copyright © 1972, The American Society for Clinical Investigation
Abstract
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holesteryl ester storage disease has been shown to involve severe deficiency of acid cholesteryl ester hydrolase and triglyceride lipase activity in liver, spleen, and lymph node. The cholesteryl ester hydrolase was also deficient in aorta. Tissue storage of both cholesteryl esters and triglycerides is generalized. Both the lipid and enzymatic changes are very similar to those in Wolman's disease.
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