Abstract
In patients with fructose-1,6-bisphosphatase (FBPase) deficiency, maternal and fetal neurodevelopmental abnormalities can be induced by severe hypoglycemia and lactic acidosis, which is triggered by pregnancy. The patient was a 24-year-old and primipara. Via pre-pregnancy education and management including self-monitoring of blood glucose and prompt medical intervention with glucose infusions, she never experienced hypoglycemia with impaired consciousness. She delivered a 2,406 g male infant at 38 weeks of gestation by cesarean section. No neurological abnormalities in either the mother or the child appeared during the following 2 years. This is the first report with detailed descriptions of the management of a pregnant woman with FDPase deficiency. This report suggests that for a patient with FBPase deficiency, the maternal and fetal complications can be reduced by pre-pregnancy education and self-monitoring of blood glucose to prevent hypoglycemia.
Competing interests: None declared
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The authors thank their patient for permission to report her case.
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Communicated by: Alberto B Burlina, MD
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Synopsis
For a patient with fructose-1,6-bisphosphatase deficiency, the maternal and fetal complications can be reduced by pre-pregnancy education and self-monitoring of blood glucose to prevent hypoglycemia.
Conflict of Interest
Genki Sugita, Hideaki Tsuyoshi, Koji Nishijima, and Yoshio Yoshida declare that they have no conflict of interest.
Informed Consent
All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2000 (5). Informed consent was obtained from all patients for being included in the study.
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This report does not contain any studies with animal subjects performed by any of the authors.
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I acknowledge that all authors have contributed significantly to the planning, conduct, and reporting of the manuscript, and that all authors are in agreement with the content of the manuscript. The guarantor for this report is the corresponding author, Hideaki Tsuyoshi, M.D.
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Sugita, G., Tsuyoshi, H., Nishijima, K., Yoshida, Y. (2014). Fructose-1,6-Bisphosphatase Deficiency: A Case of a Successful Pregnancy by Closely Monitoring Metabolic Control. In: Zschocke, J., Gibson, K., Brown, G., Morava, E., Peters, V. (eds) JIMD Reports, Volume 14. JIMD Reports, vol 14. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2013_290
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DOI: https://doi.org/10.1007/8904_2013_290
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