Decrease of Urinary Acid Mucopolysaccharides in Hurler's Syndrome after Plasma Infusion

Influence of Fresh Plasma Infusions on Patients with Genetic Mucopolysaccharidosis

“…Furthermore, Booth and Nadler (1973) reported that early in the course of therapy, high-molecular weight urinary acid mucopolysaccharides dropped precipitously, and low molecular weight breakdown products increased upon infu sion of plasma; however, both returned to preinfusion levels within 24 hr of discontinuing plasma infusion. The previous (Yamaguti et al 1973;Orii et al 1974b) and present work confirmed that our patients (especially Cases 2-5) showed remarkable transient changes in excretion of CPC-precipitable and non-CPCprecipitable substances in response to infusion of fresh plasma. The changes were more marked and of longer duration than those reported by other workers (Dekaban et al 1972;Erickson et al 1972;Danes et al 1973) with the exception of the data reported by DiFerrante et al (1971).…”

“…The present paper is a report of a detailed study of the influence of fresh plasma infusions to five patients with mucopolysacchari dosis. A part of the data presented has been described preliminarily (Orii et al 1974b). …”