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Wolman Disease MeSH Descriptor Data 2021


MeSH Heading
Wolman Disease
Tree Number(s)
C16.320.565.398.641.201.500
C16.320.565.595.201.500
C16.614.947
C18.452.584.687.201.500
C18.452.648.398.641.201.500
C18.452.648.595.201.500
Unique ID
D015223
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D015223
Scope Note
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.
Entry Version
WOLMAN DIS
Entry Term(s)
Acid Cholesteryl Ester Hydrolase Deficiency, Wolman Type
Acid Lipase Deficiency
Familial Xanthomatosis
Liposomal Acid Lipase Deficiency, Wolman Type
Wolman's Disease
Xanthomatosis, Familial
Xanthomatosis, Wolman's
Previous Indexing
Cholesterol Esters (1978-1988)
Lipase (1966-1988)
Lipid Metabolism, Inborn Errors (1966-1988)
Lipoidosis (1966-1988)
Xanthomatosis (1966-1988)
Public MeSH Note
1989
History Note
1989
Date Established
1989/01/01
Date of Entry
1988/05/12
Revision Date
2013/07/08
Wolman Disease Preferred
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