The severe infantile form of inherited lysosomal lipid storage diseases due to deficiency of acid lipase (STEROL ESTERASE). It is characterized by the accumulation of neutral lipids, particularly CHOLESTEROL ESTERS in leukocytes, fibroblasts, and hepatocytes. It is also known as Wolman's xanthomatosis and is an allelic variant of CHOLESTEROL ESTER STORAGE DISEASE.
Familjär xantomatos — Lysosomal syralipasbrist, Wolman typ
Disease, Wolman — Acid Cholesteryl Ester Hydrolase Deficiency, Wolman Type — Acid Lipase Deficiency — Acid Lipase Deficiencies — Deficiencies, Acid Lipase — Deficiency, Acid Lipase — Lipase Deficiencies, Acid — Lipase Deficiency, Acid — Cholesterol ester hydrolase deficiency — Familial Xanthomatosis — Familial Xanthomatoses — Xanthomatoses, Familial — LAL Deficiency — Deficiencies, LAL — Deficiency, LAL — LAL Deficiencies — LIPA Deficiency — Deficiencies, LIPA — Deficiency, LIPA — LIPA Deficiencies — Liposomal Acid Lipase Deficiency, Wolman Type — Lysosomal Acid Lipase Deficiency — Wolman Disease with Hypolipoproteinemia and Acanthocytosis — Wolman's Disease — Disease, Wolman's — Wolmans Disease — Xanthomatosis, Familial — Xanthomatosis, Wolman's — Wolman's Xanthomatosis — Xanthomatosis, Wolman — Xanthomatosis, Wolmans — Acid Cholesteryl Ester Hydrolase Deficiency, Type 2 — Acid Lipase Disease — Acid Lipase Diseases — Disease, Acid Lipase — Diseases, Acid Lipase — Lipase Disease, Acid — Lipase Diseases, Acid