Document Detail

Metabolic control of von Gierke disease (glycogen storage disease type Ia) in pregnancy: maintenance of euglycemia with cornstarch.
MedLine Citation:
PMID:  2304723     Owner:  NLM     Status:  MEDLINE    
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.
M P Johnson; A Compton; A Drugan; M I Evans
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Obstetrics and gynecology     Volume:  75     ISSN:  0029-7844     ISO Abbreviation:  Obstet Gynecol     Publication Date:  1990 Mar 
Date Detail:
Created Date:  1990-03-20     Completed Date:  1990-03-20     Revised Date:  2009-10-26    
Medline Journal Info:
Nlm Unique ID:  0401101     Medline TA:  Obstet Gynecol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  507-10     Citation Subset:  AIM; IM    
Department of Obstetrics/Gynecology, Wayne State University/Hutzel Hospital, Detroit, Michigan.
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MeSH Terms
Blood Glucose / analysis*
Glycogen Storage Disease Type I / blood,  therapy*
Infant, Newborn
Pregnancy Complications / blood,  therapy*
Pregnancy, Multiple
Starch / therapeutic use*
Zea mays
Reg. No./Substance:
0/Blood Glucose; 9005-25-8/Starch

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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