Disease Symptoms/Characteristics |
Wolman Disease |
Cholesteryl Ester Storage Disease |
Lipid Profile |
Elevated LDL-cholesterol, triglycerides. Reduced levels of HDL |
Elevated LDL-cholesterol, triglycerides, serum total cholesterol [16] Reduced levels of HDL |
Liver Function |
Elevated ALT + AST |
Elevated ALT + AST |
Hepatic Pathology |
Yellow and greasy. Infiltration by lipid-filled Kupffer cells [30]. Fibrosis. |
Microvesicular steatosis of the hepatocytes. Cirrhosis. Infiltration by lipid-filled Kupffer cells and macrophages [36] |
Splenic Pathology |
Enlarged spleen [27] |
Enlarged spleen [11] |
Adrenal Pathology |
Calcification of the adrenal glands [25] |
Very rare calcification of the adrenal glands [16] |
Intestinal Pathology |
Massive CE and TG accumulation in the small intestine. Infiltration of the lamina propria by foamy macrophages [34] |
CE accumulation, but to a much lesser degree than WD [16] |
Biochemical Features |
No functioning levels of LAL [34] |
1-12% of functioning LAL relative to healthy patients [11] |
Age of Onset |
First few months of birth [34] |
Childhood to Adulthood [16] |
Life Expectancy |
<1 year [34] |
Patients live into adulthood [16] |
Causes of Death |
Malnutrition, malabsorption, liver failure [34] |
Liver failure [16] |