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What is The metabolite accumulating in Wolman&;s disease is and explain in detail?
Wolman Disease and Cholesteryl Ester Storage Disease:These autosomal recessive disorders are caused by lack of lysosomal acid lipase. After LDL cholesterol is incorporated into the cell by endocytosis,it is delivered to lysosomes where it is hydrolyzed by lysosomal lipase. Failure of hydrolysis because of the complete absence of the enzyme causes accumulation of cholesteryl esters within the cells. Hepatosplenomegaly, steatorrhea, and failure to thrive occur during early infancy, leading to death at the age of 1 yr.In cholesterol ester storage disease, a less-severe form than Wolman disease, there is low but detectable acid lipase activity.Reference: Nelson Textbook of Paediatrics; 20th edition; Chapter 86; Defects in Metabolism of Lipids