title: Metabolic control of von Gierke disease (glycogen storage disease type Ia) in pregnancy: maintenance of euglycemia with cornstarch.
abstract: In patients with glycogen storage disease type Ia, 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 Ia, 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 Ia can be safely managed in pregnancy under a tightly monitored and regulated protocol of raw cornstarch feedings.
authors.affiliation: Department of Obstetrics/Gynecology, Wayne State University/Hutzel Hospital, Detroit, Michigan.
authors.forename: M P
authors.initials: MP
authors.lastname: Johnson
authors.forename: A
authors.initials: A
authors.lastname: Compton
authors.forename: A
authors.initials: A
authors.lastname: Drugan
authors.forename: M I
authors.initials: MI
authors.lastname: Evans
mesh_terms: D000328:Adult; D001786:Blood Glucose / Q000032:analysis*; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000097:blood / Q000628:therapy*; D006801:Humans; D007231:Infant, Newborn; D011247:Pregnancy; D011248:Pregnancy Complications / Q000097:blood / Q000628:therapy*; D011272:Pregnancy, Multiple; D013213:Starch / Q000627:therapeutic use*; D014310:Triplets; D003313:Zea mays
chemical_list: D001786:Blood Glucose; D013213:Starch