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Wolman Disease (Wolman's Disease) Summary

Description: 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 Show All >>

Networked: 126 relevant articles (3 outcomes, 3 trials/studies) for this Disease

Key Drugs and Agents for Wolman Disease

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Sterol Esterase (Hormone-Sensitive Lipase) : 1 outcome in 50 results : IBA
  2. Antigens : 1 outcome in 1 result : IBA
  3. Cholesterol : 1 study in 17 results : IBA
  4. Lipids : 1 study in 11 results : IBA
  5. Acetylesterase : 1 study in 5 results : IBA
  6. Calcium : 1 study in 1 result : IBA
  7. Lipase (Acid Lipase) : 34 results : FDA 1
  8. Lysosomal acid lipase deficiency : 15 results : IBA
  9. Cholesterol Esters (Cholesteryl Esters) : 12 results : IBA
  10. Triglycerides (Triacylglycerol) : 10 results : IBA
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Diseases Related to Wolman Disease

  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)
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Key Therapies for Wolman Disease

Efficacy Chart >>
  1. Transplants (Transplant) : 1 outcome in 4 results
  2. Hematopoietic Stem Cell Transplantation : 3 results
  3. Bone Marrow Transplantation (Transplantation, Bone Marrow) : 2 results
  4. Cell Transplantation : 1 result
  5. X-Ray Film : 1 result
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