Wolmans xanthomatosis

Wolman's xanthomatosis --> cholesterol ester storage disease

A rare benign adult form of inherited lysosomal lipid storage disease that is due to deficiency of acid lipase. It results in an accumulation of neutral lipids, particularly cholesterol esters, within cells (particularly leukocytes, fibroblasts, and liver cells). It is an allelic variant of wolman disease.

Please contribute to this project, if you have more information about this term feel free to edit this page

This page was last modified on 3 October 2005, at 21:16. This page has been accessed 780 times. 
What links here | Related changes | Permanent link