"Wolman Disease" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
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.
| Descriptor ID |
D015223
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| MeSH 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
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| Concept/Terms |
Wolman Disease- Wolman Disease
- Disease, Wolman
- Acid Lipase Deficiency
- Xanthomatosis, Wolman's
- Wolman's Xanthomatosis
- Xanthomatosis, Wolman
- Xanthomatosis, Wolmans
- Liposomal Acid Lipase Deficiency, Wolman Type
- Wolman's Disease
- Disease, Wolman's
- Wolmans Disease
- Xanthomatosis, Familial
- Familial Xanthomatoses
- Xanthomatoses, Familial
- Acid Cholesteryl Ester Hydrolase Deficiency, Wolman Type
- Familial Xanthomatosis
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Below are MeSH descriptors whose meaning is more general than "Wolman Disease".
Below are MeSH descriptors whose meaning is more specific than "Wolman Disease".
This graph shows the total number of publications written about "Wolman Disease" by people in this website by year, and whether "Wolman Disease" was a major or minor topic of these publications.
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| Year | Major Topic | Minor Topic | Total |
|---|
| 2009 | 1 | 0 | 1 |
| 2015 | 1 | 0 | 1 |
| 2016 | 1 | 0 | 1 |
| 2019 | 1 | 0 | 1 |
| 2022 | 1 | 0 | 1 |
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Below are the most recent publications written about "Wolman Disease" by people in Profiles.
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Structure-based virtual screening to identify potential lipase inhibitors to reduce lipid storage in Wolman disorder. Adv Protein Chem Struct Biol. 2023; 133:351-363.
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Opening a window on lysosomal acid lipase deficiency: Biochemical, molecular, and epidemiological insights. J Inherit Metab Dis. 2019 05; 42(3):509-518.
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Lysosomal Acid Lipase Deficiency Unmasked in Two Children With Nonalcoholic Fatty Liver Disease. Pediatrics. 2016 10; 138(4).
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Lysosomal Acid Lipase Deficiency (LALD). Congenit Heart Dis. 2015 May-Jun; 10(3):191-2.
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Pathological evidence of Wolman's disease following hematopoietic stem cell transplantation despite correction of lysosomal acid lipase activity. Bone Marrow Transplant. 2009 Oct; 44(7):449-50.