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Wolman Disease

More information in Books or onNLM PubMed
Definition: 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.      Other names Xanthomatosis, Wolman's; Xanthomatosis, Familial; Acid Cholesteryl Ester Hydrolase Deficiency, Wolma; Xanthomatosis, Wolmans; Xanthomatosis, Wolman; Xanthomatoses, Familial; Wolmans Disease; Wolman's Xanthomatosis; Familial Xanthomatosis; Familial Xanthomatoses; Disease, Wolman's; Disease, Wolman; Wolman's Disease; Liposomal Acid Lipase Deficiency, Wolman Type
 
SubstanceCAS Registry & nameCategoriesSource
Lysosomal acid lipase deficiency  0   *Wolman Disease.

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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