 Cholesteryl ester storage disease CESD is a rare genetic disorder characterized by deficiency or inactivity of an enzyme known as lysosomal acid lipase LIPA. This enzyme is required to breakdown metabolize certain fats lipids in the body. Deficiency of the LIPA enzyme causes these fats to abnormally accumulate in the tissues and organs of the body potentially causing a variety of symptoms. The symptoms of CESD can vary greatly depending on how much residual enzyme activity remains. The key finding and sometimes the only clinical sign is an abnormally enlarged liver hepatomegaly. Some individuals may not be diagnosed with CESD until adulthood. CESD is caused by different mutations of the same gene lysosomal acid lipase LIPA gene that causes Wolman disease. CESD is inherited as an autosomal recessive trait. 