| Three consecutive pregnancies in a patient with glycogen storage disease type IA (von Gierke's disease). | |
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MedLine Citation:
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PMID: 8203427 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Glycogen storage disease type IA is associated with metabolic abnormalities that can compromise fetal outcome. Normal outcome can be achieved by maintaining euglycemia throughout gestation. We report three consecutive pregnancies in a patient with glycogen storage disease type IA. The patient, a 35-year-old woman, has been maintained on a regimen of nightly nasogastric or cornstarch feedings for the past 12 years with improving metabolic control, reduced liver size, and no progression of multiple hepatic adenomas. On confirmation of each pregnancy, early in the first trimester nightly feeding was changed from cornstarch ingestion to Polycose by nasogastric intubation, with good metabolic control. During the last trimester of each pregnancy metabolic control showed further improvement, with lowering of lactate, urate, and triglyceride levels. During the first pregnancy unexpected fetal death occurred at 33 weeks. During the last two pregnancies, the patient was admitted at 33 and 34 weeks, respectively, for closer supervision of metabolic status and fetal monitoring. She underwent a cesarean section at 35 weeks 4 days of gestation and was delivered of a girl. She underwent a repeat cesarean section at 35 weeks 2 days for the subsequent gestation and was delivered of a boy. Both infants are healthy and appear to be unaffected by von Gierke's disease. Hepatic adenomas did not enlarge during the pregnancies. Meticulous management resulted in normal pregnancy outcomes in two consecutive gestations. Rapid fetal growth late in the third trimester may require particularly careful supervision to maintain euglycemia. |
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Authors:
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I P Ryan; R J Havel; R K Laros |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: American journal of obstetrics and gynecology Volume: 170 ISSN: 0002-9378 ISO Abbreviation: Am. J. Obstet. Gynecol. Publication Date: 1994 Jun |
Date Detail:
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Created Date: 1994-07-07 Completed Date: 1994-07-07 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 0370476 Medline TA: Am J Obstet Gynecol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 1687-90; discussion 1690-1 Citation Subset: AIM; IM |
Affiliation:
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Department of Obstetrics, Gynecology, and Reproductive Sciences, University of California, San Francisco 94143-0556. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Dietary Carbohydrates / therapeutic use Female Fetal Death Glucans / therapeutic use Glucose / administration & dosage Glycogen Storage Disease Type I / diet therapy* Humans Pregnancy Pregnancy Complications / diet therapy* Starch / therapeutic use |
| Chemical | |
Reg. No./Substance:
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0/Dietary Carbohydrates; 0/Glucans; 50-99-7/Glucose; 9005-25-8/Starch |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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