Plasma Infusions in the Hurler Syndrome

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Abstract
An attempt has been made to determine if plasma infusions begun in the neonatal period influence the course of the Hurler syndrome. The progression of the disorder was assessed in terms of clinical and developmental standards and mucopolysaccharide metabolism. Weekly infusions of plasma have been associated with a progression of the physical stigmata of the Hurler syndrome and abnormal psychomotor development at 1 year of age. There was no significant change in urinary excretion of either the total amount or the ratio of large molecular weight mucopolysaccharides to fragments during daily or weekly plasma infusions. There is no evidence that plasma, in the quantities infused, alters the development of the clinical phenotype of the Hurler syndrome.

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