LIPA and lysosomal acid lipase deficiency: Lysosomal acid lipase deficiency (LAL-D) is a lysosomal storage disorder caused by mutations in the lipase A (LIPA) gene that result in a failure of the LAL enzyme to sufficiently hydrolyze cholesteryl esters and triglycerides into free cholesterol and free fatty acids in the lysosome.1, 2, 3, 4 LAL occupies a critical and essential position in the control of plasma lipoprotein levels and in the prevention of cellular lipid overload, especially in the liver and spleen.2