Structured Summary
Abstract
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.
MeSH Record
Classification
Broader headings
Related Concepts
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MeSH Record
Synonyms
38 entry terms
- Acid Cholesteryl Ester Hydrolase Deficiency, Wolman Type
- Acid Lipase Deficiency
- Cholesterol ester hydrolase deficiency
- Familial Xanthomatosis
- LAL Deficiency
- LIPA Deficiency
- Liposomal Acid Lipase Deficiency, Wolman Type
- Lysosomal Acid Lipase Deficiency
- Wolman Disease with Hypolipoproteinemia and Acanthocytosis
- Wolman's Disease
- Xanthomatosis, Familial
- Xanthomatosis, Wolman's
- Acid Lipase Deficiencies
- Deficiencies, Acid Lipase
- Deficiencies, LAL
- Deficiencies, LIPA
- Deficiency, Acid Lipase
- Deficiency, LAL
- Deficiency, LIPA
- Disease, Wolman
- Disease, Wolman's
- Familial Xanthomatoses
- LAL Deficiencies
- LIPA Deficiencies
- Lipase Deficiencies, Acid
- Lipase Deficiency, Acid
- Wolman's Xanthomatosis
- Wolmans Disease
- Xanthomatoses, Familial
- Xanthomatosis, Wolman
- Xanthomatosis, Wolmans
- Acid Cholesteryl Ester Hydrolase Deficiency, Type 2
- Acid Lipase Disease
- Acid Lipase Diseases
- Disease, Acid Lipase
- Diseases, Acid Lipase
- Lipase Disease, Acid
- Lipase Diseases, Acid
MeSH Record
Aspects Covered
34 allowable subheadings
Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.
MeSH Record
History Note
1989
MeSH Record
Previous Indexing
- Cholesterol Esters (1978-1988)
- Lipase (1966-1988)
- Lipid Metabolism, Inborn Errors (1966-1988)
- Lipoidosis (1966-1988)
- Xanthomatosis (1966-1988)
MeSH Hierarchy
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AMA Style
References
- National Library of Medicine. Wolman Disease. Medical Subject Headings (MeSH). 2026. Unique ID D015223. http://id.nlm.nih.gov/mesh/2026/D015223
- Wolman Disease. In: Wikipedia. https://en.wikipedia.org/wiki/Lysosomal_acid_lipase_deficiency
- Wolman Disease. In: Wikidata. https://www.wikidata.org/wiki/Q6710283