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Wolman Disease (Wolman's Disease)

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.
Also Known As:
Wolman's Disease; Liposomal Acid Lipase Deficiency, Wolman Type; Disease, Wolman; Disease, Wolman's; Familial Xanthomatoses; Familial Xanthomatosis; Wolman's Xanthomatosis; Wolmans Disease; Xanthomatoses, Familial; Xanthomatosis, Wolman; Xanthomatosis, Wolmans; Acid Cholesteryl Ester Hydrolase Deficiency, Wolman Type; Xanthomatosis, Familial; Xanthomatosis, Wolman's
Networked: 126 relevant articles (3 outcomes, 3 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Cholesterol Ester Storage Disease
2. Hypercholesterolemia
3. Aortic Valve Stenosis (Aortic Stenosis)
4. Gaucher Disease (Gaucher's Disease)
5. Lysosomal Storage Diseases (Lysosomal Storage Disease)

Experts

1. Lohse, P: 3 articles (10/2013 - 01/2000)
2. Valles-Ayoub, Yadira: 2 articles (03/2015 - 06/2011)
3. Han, Seungil: 1 article (06/2015)
4. Rajamohan, Francis: 1 article (06/2015)
5. Tu, Meihua: 1 article (06/2015)
6. Ruangsiriluk, Wanida: 1 article (06/2015)
7. Kurumbail, Ravi G: 1 article (06/2015)
8. Reyes, Allan R: 1 article (06/2015)
9. Hoth, Lise R: 1 article (06/2015)
10. Caspers, Nicole: 1 article (06/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Wolman Disease:
1. Sterol Esterase (Hormone-Sensitive Lipase)IBA
2. AntigensIBA
3. CholesterolIBA
4. LipidsIBA
5. AcetylesteraseIBA
6. CalciumIBA
7. Lipase (Acid Lipase)FDA Link
8. Lysosomal acid lipase deficiencyIBA
9. Cholesterol Esters (Cholesteryl Esters)IBA
10. Triglycerides (Triacylglycerol)IBA

Therapies and Procedures

1. Transplants (Transplant)
2. Hematopoietic Stem Cell Transplantation
3. Bone Marrow Transplantation (Transplantation, Bone Marrow)
4. Cell Transplantation
5. X-Ray Film