The bulletin of the Yamaguchi Medical School

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The bulletin of the Yamaguchi Medical School Volume 21 Issue 1-4
published_at 1974-12

Two Cases in Glycogenosis Type Ⅳ.Ⅰ. Light Microscopic Observation.

Two Cases in Glycogenosis Type Ⅳ.Ⅰ. Light Microscopic Observation.
Ishihara Tokuhiro
Uchino Fumiya
Adachi Hironobu
Watanabe Seishiro
Onishi Hiromi
Ikee Yoshihiko
Misawa Sachiyo
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Descriptions
glycogenosis type Ⅳ, which is characterized by deposits of polysaccharide in the liver and liver cirrhosis resembling postencratic type, were described in two children. They were siblings, had a sister with an identical disease and had unrelated parents. Furthermore, in one case in which a partial autopsy was performed, two kind of the polysaccharide were noted in the cardiac muscle, skeletal muscle, smooth muscle and reticuloendothelial cells. The one was normal glycogen and the other was not identical with normal glycogen because of its diastase resistance. The genesis of the cirrhosis may have been a reaction to the stimulation by the polysaccharide as a foreign body.