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 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.
Johnson
M P
MP
Department of Obstetrics/Gynecology, Wayne State University/Hutzel Hospital, Detroit, Michigan.
Compton
A
A
Drugan
A
A
Evans
M I
MI
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
D001786:Blood Glucose; D013213:Starch