Wolman-disease meaning

wôl mən
A lysosomal storage disease caused by deficiency of an enzyme that breaks down lipids, characterized by the accumulation of cholesterol esters and triglycerides in the tissues and resulting in enlargement of the liver and spleen, abdominal distension, steatorrhea, cachexia, and usually death in infancy.
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A rare genetic disorder caused by deficiency of an enzyme known as lysosomal acid lipase (LAL or LIPA), necessary to break down certain lipids.
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