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May 1966

Glycogen Storage Disease, Types II, III, VIII, and IX: A Biochemical and Electron Microscopic Analysis

Author Affiliations

From the Children's Hospital Clinical Research Center and the departments of pediatrics and pathology, University of Cincinnati College of Medicine.

Am J Dis Child. 1966;111(5):457-474. doi:10.1001/archpedi.1966.02090080035001

CORI DIVIDED the syndrome of glycogen storage disease into various types according to the enzymatic deficiency involved.1 This classification has three immediate advantages. It rests on the most basic, most precise, and least overlapping parameter known at the present time. It permits delineation of a clinical picture indicative of a particular type. It allows the addition of newly recognized types without reshuffling of the whole system.

Table 1 summarizes the current state of this classification. Types I through VI of this classification are already widely accepted. Type VII comprises deficiencies of phosphoglucomutase. This enzyme's activity was found decreased in the skeletal muscle of one patient.2 In another study a patient is mentioned with a low level of phosphoglucomutase activity in both muscle and liver.3 Table 1 further includes as types VIII and IX our patients described below, whose low liver phosphorylase activity seems the result of different