DO_rare_slim NCIthesaurus lysosomal acid lipase deficiency Wolman disease DOID:14497 Acid esterase deficiency ICD10CM:E75.5 SNOMEDCT_US_2025_09_01:82500001 MESH:D015223 MIM:620151 ORDO:75233 disease_ontology ICD10CM:E75.5 GARD:7899 UMLS_CUI:C0043208 NCI:C61271 MIM:620151 ORDO:75233 A lysosomal acid lipase deficiency characterized by infantile onset of rapidly progressive accumulation of cholesteryl esters and triglycerides throughout the body, resulting in hepatosplenomegaly, severe malnutrition, jaundice, vomiting, diarrhea, steatorrhea. Death usually occurs within the first year of life. Acid lipase deficiency Wolman xanthomatosis Wolman's disease Wolman's or triglyceride storage type III disease Xanthomatosis, familial acute infantile lysosomal acid lipase deficiency complete LAL deficiency complete LIPA deficiency complete cholesterol ester hydrolase deficiency complete lysosomal acid lipase deficiency