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fucosidosis

   Also found in: Wikipedia 0.26 sec.
fucosidosis /fu·co·si·do·sis/ (fu″ko-sĭ-do´sis) a lysosomal storage disease caused by deficient enzymatic activity of fucosidase and accumulation of fucose-containing glycoconjugates in all tissues; it is marked by progressive psychomotor deterioration, growth retardation, hepatosplenomegaly, cardiomegaly, and seizures.
fu·co·si·do·sis (fy-ks-dss)
n.
An inherited metabolic storage disease caused by a deficiency of alpha-fucosidase and the accumulation of glycolipids that contain fructose, and marked by progressive neurologic deterioration, spasticity, tremor, and mild skeletal changes.

fucosidosis
[fyo̅o̅′kōsidō′sis]
a hereditary lysosomal storage disorder that results from the absence of the enzyme required to metabolize fucoside moieties. It causes mental retardation, neurologic deterioration, coarse facial features, thickened skin, and hepatosplenomegaly.


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