Charles Dent Metabolic Unit, National Hospital for Neurology and Neurosurgery, Internal Mailbox 92 Queen Square, London, WC1N 3BG, UK.
J Inherit Metab Dis. 2012 Mar;35(2):245-51. doi: 10.1007/s10545-011-9384-7. Epub 2011 Sep 7.
We present a review of our experience and pregnancy outcome in patients with GSD III managed by our centre. Between 1997 and 2010 there were 15 pregnancies in seven women with GSD III. Four women had GSD IIIb (nine pregnancies) and three GSD IIIa (six pregnancies). There was a successful outcome in all 15 pregnancies with delivery of 15 liveborn infants. Four infants were of low birthweight (<2nd centile) but all have developed normally apart from one with behavioural/psychiatric problems. Three women had pre-existing cardiomyopathy prior to pregnancy. One of these women had deterioration of her cardiomyopathy during pregnancy and again in the post-partum period. Women with GSD III do not seem to have any issues with fertility. Overall the outcome of pregnancy for both mother and child is good. Care needs to be taken to avoid maternal hypoglycemia which may be associated with intrauterine growth restriction and low birth weight. Cardiac function should be monitored carefully particularly in those with pre-existing cardiomyopathy.
我们回顾了我们中心治疗的 GSD III 患者的经验和妊娠结局。1997 年至 2010 年期间，7 名 GSD III 女性中有 15 例妊娠。4 名女性患有 GSD IIIb（9 例妊娠），3 名患有 GSD IIIa（6 例妊娠）。所有 15 例妊娠均成功，分娩出 15 名活产婴儿。4 名婴儿出生体重较低（<第 2 百分位），但除 1 名有行为/精神问题外，均发育正常。3 名妇女在妊娠前已有心肌病。其中 1 名妇女在妊娠期间和产后期间其心肌病恶化。GSD III 女性似乎没有任何生育问题。总体而言，母婴妊娠结局良好。需要注意避免母亲低血糖，低血糖可能与宫内生长受限和低出生体重有关。应特别注意那些已有心肌病的患者的心脏功能。
