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强直性肌营养不良与妊娠

发表者:吴士文 人已读

强直性肌营养不良(DM)与妊娠

       强直性肌营养不良是成人中是最常见的神经肌肉病,患病率在2.4-2.5/100000人中。作者在这里报道了2例DM患者在妊娠中出现的并发症。病例1是一位39岁的经产妇,抱怨反复腹痛,羊水过多,产后出血。以上这些症状考虑为DM的并发征。病例2是例27岁的产妇,双绒(毛)膜,双羊膜腔妊娠双胞胎。在11周时取绒毛膜基因检查,发现一个胎儿也是DM。因此采取了选择性的终止妊娠。不幸的是,出现了严重的羊水过少,慢性羊水漏,这导致在第5天后第二个胎儿宫内死亡。武警总医院神经内科吴士文

这说明在DM中给予产前诊断及快速的基因诊断是很重要的,另外,对于DM妊娠的产妇的处理应该给予有多学科参加的团队是很重要的。

 In our second case we discuss the management of a 27 year old woman with dichorionic, di-amniotic twins. Chorionic Villous Sampling at 11 weeks revealed one of the fetuses, a male; to be afflicted by DM. Selective termination of the affected twin was performed. Unfortunately, she developed severe oligohydramnios and chronic liquor leak. This resulted in the intra-uterine death of the second twin 5 days later. Our cases highlight the importance of prenatal diagnosis and prompt genetic counselling. A multidisciplinary team approach is required in the management of such high risk cases.

J Pak Med Assoc. 2009 Oct;59(10):717-9.

Myotonic dystrophy and pregnancy.

Khan ZA, Khan SA.

West Cumberland Hospital, Whitehaven, United Kingdom.

Myotonic dystrophy is the most common neuromuscular disease in adults with a prevalence of 2.4-5.5 per 100,000. Here we describe two cases of DM and discuss their obstetric complications. Our first case concerns a 39 year old multipara whose pregnancies were complicated by recurrent abdominal pain, polyhydramnios and post partum haemorrhage which was attributed to DM. In our second case we discuss the management of a 27 year old woman with dichorionic, di-amniotic twins. Chorionic Villous Sampling at 11 weeks revealed one of the fetuses, a male; to be afflicted by DM. Selective termination of the affected twin was performed. Unfortunately, she developed severe oligohydramnios and chronic liquor leak. This resulted in the intra-uterine death of the second twin 5 days later. Our cases highlight the importance of prenatal diagnosis and prompt genetic counselling. A multidisciplinary team approach is required in the management of such high risk cases.

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发表于:2009-12-19 10:44

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吴士文的咨询范围: 神经肌肉病,肌营养不良,遗传病,

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