| Descriptor English: | Wolman Disease | ||||||
| Descriptor Spanish: |
Enfermedad de Wolman
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| Descriptor Portuguese: | Doença de Wolman | ||||||
| Descriptor French: | Maladie de Wolman | ||||||
| Entry term(s): |
Acid Cholesteryl Ester Hydrolase Deficiency, Type 2 Acid Cholesteryl Ester Hydrolase Deficiency, Wolman Type Acid Lipase Deficiency Acid Lipase Disease Acid Lipase Diseases Cholesterol ester hydrolase deficiency Deficiencies, LAL Deficiencies, LIPA Deficiency, LAL Deficiency, LIPA Disease, Acid Lipase Disease, Wolman Disease, Wolman's Diseases, Acid Lipase Familial Xanthomatoses Familial Xanthomatosis LAL Deficiencies LAL Deficiency LIPA Deficiencies LIPA Deficiency Lipase Disease, Acid Lipase Diseases, Acid Liposomal Acid Lipase Deficiency, Wolman Type Lysosomal Acid Lipase Deficiency Wolman Disease with Hypolipoproteinemia and Acanthocytosis Wolman's Disease Wolman's Xanthomatosis Wolmans Disease Xanthomatoses, Familial Xanthomatosis, Familial Xanthomatosis, Wolman Xanthomatosis, Wolman's Xanthomatosis, Wolmans |
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| Tree number(s): |
C16.320.565.398.641.201.500 C16.320.565.595.201.500 C16.614.947 C18.452.584.563.641.201.500 C18.452.648.398.641.201.500 C18.452.648.595.201.500 |
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| RDF Unique Identifier: | https://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. |
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| Allowable Qualifiers: |
BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications DG diagnostic imaging DH diet therapy DI diagnosis DT drug therapy EC economics EH ethnology EM embryology EN enzymology EP epidemiology ET etiology GE genetics HI history IM immunology ME metabolism MI microbiology MO mortality NU nursing PA pathology PC prevention & control PP physiopathology PS parasitology PX psychology RH rehabilitation RT radiotherapy SU surgery TH therapy UR urine VE veterinary VI virology |
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| Previous Indexing: |
Cholesterol Esters (1978-1988) Lipase (1966-1988) Lipid Metabolism, Inborn Errors (1966-1988) Lipoidosis (1966-1988) Xanthomatosis (1966-1988) |
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| Public MeSH Note: | 1989 |
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| History Note: | 1989 |
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| Entry Version: | WOLMAN DIS |
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| DeCS ID: | 23863 | ||||||
| Unique ID: | D015223 | ||||||
| Documents indexed in the Virtual Health Library (VHL): | Click here to access the VHL documents | ||||||
| Date Established: | 1989/01/01 | ||||||
| Date of Entry: | 1988/05/12 | ||||||
| Revision Date: | 2023/05/31 |
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Wolman Disease
- Preferred
Lysosomal Acid Lipase Deficiency
- Narrower
| Concept UI |
M0023386 |
| 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. |
| Preferred term | Wolman Disease |
| Entry term(s) |
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 Disease with Hypolipoproteinemia and Acanthocytosis Wolman's Disease Wolman's Xanthomatosis Wolmans Disease Xanthomatoses, Familial Xanthomatosis, Familial Xanthomatosis, Wolman Xanthomatosis, Wolman's Xanthomatosis, Wolmans |
| Concept UI |
M0525345 |
| Preferred term | Lysosomal Acid Lipase Deficiency |
| Entry term(s) |
Acid Cholesteryl Ester Hydrolase Deficiency, Type 2 Acid Lipase Disease Acid Lipase Diseases Cholesterol ester hydrolase deficiency Deficiencies, LAL Deficiencies, LIPA Deficiency, LAL Deficiency, LIPA Disease, Acid Lipase Diseases, Acid Lipase LAL Deficiencies LAL Deficiency LIPA Deficiencies LIPA Deficiency Lipase Disease, Acid Lipase Diseases, Acid |
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