| Pregnancy in glycogen storage disease type Ib: gestational care and report of first successful deliveries. | |
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MedLine Citation:
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PMID: 20386986 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Patients with type Ia glycogen storage disease (GSD) have been surviving well into adulthood since continuous glucose therapy was introduced in the 1970s, and there have been many documented successful pregnancies in women with this condition. Historically, few individuals with type Ib GSD, however, survived into adulthood prior to the introduction of granulocyte colony stimulating factor (G-CSF) in the late 1980s. There are no previously published reports of pregnancies in GSD type Ib. In this case report we describe the course and management of five successful pregnancies in three patients with GSD type Ib. Patient 1 experienced an increase in glucose requirement in all three of her pregnancies, starting from the second trimester onwards. There were no major complications related to neutropenia except for oral ulcers. The infants did well, except for respiratory distress in two of them at birth. Patient 2 used cornstarch to maintain euglycemia, but precise dosing was not part of her regimen, and, hence, an increase in metabolic demands was difficult to demonstrate. She developed a renal calculus and urinary tract infection during her pregnancy and had chronic iron deficiency anemia but no neutropenia. The neonate did well after delivery. Patient 3 had poor follow-up during pregnancy. Increasing glucose requirements, worsening lipid profile, neutropenia associated with multiple infections, and anemia were noted. The newborn infant did well after delivery. In addition to the case reports, the challenges of the usage of G-CSF, the treatment of enterocolitis, and comparisons with the management of GSD Ia are discussed. |
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Authors:
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Aditi I Dagli; Philip J Lee; Catherine E Correia; Christina Rodriguez; Kaustav Bhattacharya; Linda Steinkrauss; Charles A Stanley; David A Weinstein |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2010-4-13 |
Journal Detail:
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Title: Journal of inherited metabolic disease Volume: - ISSN: 1573-2665 ISO Abbreviation: - Publication Date: 2010 Apr |
Date Detail:
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Created Date: 2010-4-13 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7910918 Medline TA: J Inherit Metab Dis Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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Glycogen Storage Disease Program and Division of Pediatric Endocrinology, University of Florida College of Medicine, P.O. Box 100296, Gainesville, FL, 32610-0296, USA, adagli@peds.ufl.edu. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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