Metabolic control of von gierke disease (glycogen storage disease type ia) in pregnancy: Maintenance of euglycemia with cornstarch

In patients with glycogen storage disease type la, glucose-6-phosphatase deficiency reduces the liver’s ability to generate free glucose from glycogen. Without a continuous, exogenous source of glucose, severe hypoglycemia and subsequent metabolic perturbations occur. Our observations of a patient with glycogen storage disease type la, who also had a clomiphene-induced triplet gestation, suggest that cornstarch, which can be catabolized by debranching enzymes, may be used to maintain a constant state of maternal and fetal euglycemia and correct many metabolic abnormalities. Our data suggest that patients with glycogen storage disease type la can be safely managed in pregnancy under a tightly monitored and regulated protocol of raw cornstarch feedings.