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JIMD Rep. 2014;14:115-8. doi: 10.1007/8904_2013_290. Epub 2014 Jan 28.

Fructose-1,6-bisphosphatase deficiency: a case of a successful pregnancy by closely monitoring metabolic control.

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Faculty of Medical Sciences, Department of Obstetrics and Gynecology, University of Fukui, 23-3 Matsuoka-Shimoaizuki, Eiheiji-cho, Yoshida-Gun, 910-1193, Fukui, Japan.


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.

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