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首页> 外文期刊>The Journal of perinatal & neonatal nursing >Pregnancy in women with glycogen storage disease Ia and Ib
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Pregnancy in women with glycogen storage disease Ia and Ib

机译：糖原贮积病Ia和Ib的女性怀孕

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Over the past 9 decades since glycogen storage disease (GSD) was described, an almost universally fatal disease has become one where women are living well into adulthood and choosing to bear children. This inborn error of metabolism associated with the creation and utilization of glycogen, when untreated, manifests with unrelenting hypoglycemia. The initiation of continuous feeds has improved outcomes, and later in 1982, the administration of intermittent doses of cornstarch in water provided a continuous supply of exogenous glucose. As metabolic control has improved, morbidity has decreased. Glycogen storage disease Ib has the same severity of hypoglycemia as GSD Ia, with associated immune disturbance. Prior to the introduction of granulocyte colony-stimulating factor (G-CSF), infections caused significant mortality in GSD Ib. Pregnancy in patients with GSD Ia and Ib poses unique challenges during gestation and delivery. Good metabolic control before conception and throughout pregnancy is directly related to successful outcomes. There is no nursing literature to date addressing perinatal and neonatal care in this population.
机译：自从糖原贮积病（GSD）被描述以来，在过去的九十年中，一种几乎普遍致命的疾病已经成为妇女成年后生活良好并选择生育的疾病。如果不加以治疗，这种与糖原的产生和利用有关的先天性代谢错误表现为无休止的低血糖症。连续喂食的开始改善了结局，1982年下半年，间歇剂量的玉米淀粉在水中的施用为外源性葡萄糖提供了持续供应。随着代谢控制的改善，发病率降低了。糖原贮积病Ib的低血糖严重程度与GSD Ia相同，并伴有免疫紊乱。在引入粒细胞集落刺激因子（G-CSF）之前，感染引起了GSD Ib的重大死亡。 GSD Ia和Ib患者的妊娠在妊娠和分娩期间提出了独特的挑战。受孕前和整个妊娠期间良好的代谢控制与成功的结果直接相关。迄今为止，尚无有关该人群围产期和新生儿护理的护理文献。

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