primary familial xanthomatosis

primary familial xanthomatosis
Wolman xanthomatosis Wolman disease.

Medical dictionary. 2011.

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  • Wolman disease — a lysosomal storage disease caused by deficiency of the lysosomal sterol esterase, with onset in early infancy and death before one year of age. Clinical features include hepatosplenomegaly, steatorrhea, abdominal distention, anemia, inanition,… …   Medical dictionary

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