Acid lipase disease
Acid Lipase Disease
Acid Lipase Disease (pronounced as: a-sid li-peys di-zeez) is a collective term for two rare genetic disorders, Wolman disease and Cholesteryl ester storage disease (CESD). These diseases are characterized by the buildup of fats in the body due to the deficiency of an enzyme known as lysosomal acid lipase (LAL).
Etymology
The term "Acid Lipase Disease" is derived from the enzyme that is deficient in these conditions, "acid lipase". Acid lipase is responsible for breaking down certain fats in the body. When this enzyme is deficient, it leads to the diseases known as Acid Lipase Disease.
Wolman Disease
Wolman disease (pronounced as: wool-man di-zeez) is a severe form of Acid Lipase Disease that typically presents in infancy. It is characterized by the accumulation of fats in the liver, spleen, and other tissues, leading to liver failure and death usually within the first year of life.
Cholesteryl Ester Storage Disease
Cholesteryl ester storage disease (CESD) (pronounced as: ko-les-ter-il es-ter stor-age di-zeez) is a milder form of Acid Lipase Disease. It is characterized by the accumulation of fats in the liver, leading to liver disease and other complications. The onset and severity of CESD can vary widely, from childhood to adulthood.
Related Terms
- Lysosomal acid lipase deficiency (LAL-D) (pronounced as: li-so-so-mal a-sid li-peys di-fi-shen-see) is the medical term for the deficiency of the enzyme lysosomal acid lipase, which leads to Acid Lipase Disease.
- Lysosome (pronounced as: li-so-som) is a part of the cell that breaks down waste materials and cellular debris. Lysosomes contain enzymes, like acid lipase, that break down these materials.
- Enzyme (pronounced as: en-zym) is a type of protein that speeds up chemical reactions in the body.
External links
- Medical encyclopedia article on Acid lipase disease
- Wikipedia's article - Acid lipase disease
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