Canadian Medical Guide > Diseases > Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Genetic Diseases, Inborn > Metabolism, Inborn Errors > Lipid Metabolism, Inborn Errors > Lipoidosis Terms and Definitions

Lipoidosis

Medical Definition:	A disturbance of lipid metabolism with abnormal deposit of lipids in the cells. (Dorland, 27th ed)
Guide Notes:	an inborn error of lipid metab; do not use /congen & do not coord with INFANT, NEWBORN, DISEASES
Also Called:	Amaurotic Familial Idiocy

Lipoidosis Categories.

	Cholesterol Ester Storage Disease - A rare benign adult form of inherited lysosomal lipid storage disease that is due to deficiency of acid lipase. It results in an accumulation of neutral lipids, particularly cholesterol esters, within cells (particularly leukocytes, fibroblasts, and liver cells). It is an allelic variant of WOLMAN DISEASE.
	Lipoidproteinosis - A familial disease occurring in the course of latent diabetes, marked by yellowish nodules on the skin and mucosae, keratotic lesions on the extremities, and hoarseness due to faulty lipid metabolism. (Dorland, 27th ed)
	Wolman Disease - The severe infantile form of inherited lysosomal lipid storage diseases, due to deficiency of acid lipase. It results in accumulation of neutral lipids, particularly cholesterol esters, within cells (particularly leukocytes, fibroblasts, and liver cells). An allelic variant of CHOLESTEROL ESTER STORAGE DISEASE, it is also known as Wolman's xanthomatosis.

Lipoidosis Medical Definitions and Terms

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