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