Mutations in LIPA cause LAL deficiency, a very rare disorder with two recognized phenotypes: Wolman disease, characterized by the complete absence of LAL activity and high and premature mortality rate, and Cholesteryl Ester Storage Disease (CESD), with a residual 5–10% LAL activity accompanied by high transaminases, hepatomegaly, liver fibrosis, dyslipidemia (high LDL-C and low HDL-C), as well as premature atherosclerosis and CVD [112]. The gene discussed is LIPA; the disease is Wolman disease.