Wolman Disease


Acid Cholesteryl Ester Hydrolase Deficiency, Wolman Type

Acid Lipase Deficiency

Disease, Wolman

Disease, Wolman's

Familial Xanthomatoses

Familial Xanthomatosis

Liposomal Acid Lipase Deficiency, Wolman Type

Wolman's Disease

Wolman's Xanthomatosis

Wolmans Disease

Xanthomatoses, Familial

Xanthomatosis, Familial

Xanthomatosis, Wolman

Xanthomatosis, Wolman's

Xanthomatosis, Wolmans

The severe infantile form of inherited lysosomal lipid storage diseases due to deficiency of acid lipase (STEROL ESTERASE). It is characterized by the accumulation of neutral lipids, particularly CHOLESTEROL ESTERS in leukocytes, fibroblasts, and hepatocytes. It is also known as Wolman's xanthomatosis and is an allelic variant of CHOLESTEROL ESTER STORAGE DISEASE.