The Tohoku Journal of Experimental Medicine
Online ISSN : 1349-3329
Print ISSN : 0040-8727
ISSN-L : 0040-8727
Severe Infantile Sialidosis
The Characteristics of Oligosaccharides Isolated from the Urine and the Abdominal Ascites
YUTAKA NAKAMURAYUKITOSHI TAKAHASHISEIJI YAMAGUCHISHIROU OMIYATADAO ORIIASAO YARAMIYUKI GUSHIKEN
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1992 Volume 166 Issue 4 Pages 407-415

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Abstract

NAKAMURA, Y., TAKAHASHI, Y., YAMAGUCHI, S., OMIYA, S., ORII, T., YARA, A. and GUSHIKEN, M. Severe Infantile Sialidosis-The Characteristics of Oligosaccharides Isolated from the Urine and the Abdominal Ascites. Tohoku J. Exp. Med., 1992, 166 (4), 407-415-A female infant presenting congenital ascites, hepatosplenomegaly, coarse face, and delayed mental and physical development is described. Oligosaccharides in the urine and the abdominal ascites of this patient were investigated by Bio-Gel column chromatography. The level of urinary oligosaccharides excreted (nmol/mg creatinine) by the patient was 25.8 times the levels of the controls. The oligosaccharides in the urine and the ascites were composed almost entirely of sialyl oligosaccharides. The structures of urinary oligosaccharides were the same as those of oligosaccharides in the ascites. The ratio of high molecular weight sialyl oligosaccharides with repeating structures to low molecular weight sialyl oligosaccharides was higher in the ascites than in the urine.

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