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Successful treatment of diabetes associated with glycogen storage disease type Ia

A 22-year-old woman was diagnosed with glycogen storage disease type Ia (GSD Ia) at the age of 2.5 years. This was treated with uncooked corn starch. Twenty years later she developed diabetes with significant hyperglycaemia (fasting blood glucose 8.6 mmol/l, postprandial glucose 26 mmol/l). At presentation, she had short stature (height 1.29 m, weight 31 kg, BMI 18.5 kg/m2 ). Type 1 diabetes-related antibodies were negative. She had marginally elevated liver transaminases and hypertriglyceridemia (Table 1).