The Tohoku Journal of Experimental Medicine
Online ISSN : 1349-3329
Print ISSN : 0040-8727
ISSN-L : 0040-8727
Influence of Fresh Plasma Infusions on Patients with Genetic Mucopolysaccharidosis
TADAO ORIIMAMORU YAMAGUCHIRYOJI MINAMIHIROYOSHI ITOTOORU NAKAO
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1974 Volume 114 Issue 4 Pages 385-392

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Abstract

The pattern of urinary acid mucopoly-saccharide excretion before, during and after fresh plasma infusions to five patients with mucopolysaccharidosis has been studied using the cetylpyridinium chloride (CPC) precipitation methcd. In all of the patients (three cases with Hurler syndrome, one case with Scheie syndrome and one case with Morquio syndrome), the results obtained were summarized as follows: 1) The plasma infusions were followed by a transient decreased urinary excretion of acid mucopolysaccharides. 2) Furthermore, the infusions were followed by an increased excretion of the non-CPC-precipitates. 3) Ratios of acid mucopolysaccharides to non-CPC-precipitates showed the lowest values on the third or fourth day after plasma infusions. 4) It is suggested that plasma infusions did not modify the course of the patients with mucopolysaccharidosis.

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