Two patients who had severe unstable diabetes mellitus with frequent hypoglycemic episodes and who required soluble insulin for control are described. Both developed hepatomegaly. Quantitative and qualitative studies of liver glycogen and its related enzymes were performed. The liver glycogen content was high (8 per cent) but its molecular structure was virtually normal. Both the β-amylolysis limit and the phosphorylase activity appeared to be lower than normal but did not approach the levels associated with glycogen storage disorders. The values of the other enzymes tested were normal. Therefore it appears that the elevated liver glycogen levels seen in some patients with brittle diabetes mellitus are not the result of enzyme deficiency but are secondary to wide fluctuations in blood sugar and frequent doses of soluble insulin.

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