重症肌无力患者妊娠期临床特点及预后分析

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目的:探讨重症肌无力(MG)患者妊娠前后肌无力症状的变化及MG对妊娠预后的影响。方法:回顾性研究2013年1月至2018年10月28例就诊于解放军总医院第八医学中心MG患者38例次妊娠分娩的临床资料。比较妊娠前与妊娠不同时期MG严重程度评分、血乙酰胆碱受体(AChR)抗体变化及药物的使用情况,并分析妊娠后MG不同转归(改善组、无变化组及恶化组)与MG病程、MG严重程度、重复电刺激(RNS)、胸腺情况的相关性,同时监测妊娠、分娩及新生儿情况。结果:(1)MG妊娠时年龄为21~36(27±4)岁。MG病程为0.5~17.2(7.4±5.8)年。Osserman临床分型以ⅡA型为主,占44.1%(15/34),其次为Ⅰ型为29.4%(10/34),ⅡB型占23.5%(8/34),仅1例(2.9%)为Ⅳ型。(2)28例MG患者经38次妊娠,其中3次胎停,1次自然流产,34次活胎。34次活胎妊娠过程中,16例次(47.1%)MG症状改善,10例次(29.4%)MG症状恶化,8例次(23.5%)MG无变化。其中恶化多见于妊娠早期、晚期。(3)比较妊娠后不同MG转归发现,恶化组妊娠前MG病程短于改善组和无变化组,严重程度评分和RNS异常率高于改善组及无变化组[(1.1±0.5)年比(7.1±5.1)年、(9.0±5.4)年;(20.9±6.0)分比(14.8±6.6)分、(13.3±5.0)分;9/10例次比 8/16例次、4/8例次];临床分型来看,恶化组患者ⅡB型高于其他两组(6/10例次比1/16例次、1/8例次),差异有统计学意义(n P值均<0.01),而血AChR抗体阳性率及胸腺切除在三组中差异无统计学意义。(4)34次活胎妊娠中23次硬膜外麻醉下行剖腹产,11次经阴道分娩,新生儿未发现肢体挛缩,无一过性重症肌无力症状。n 结论:MG患者在病情控制平稳的情况下妊娠是安全的,妊娠前MG病程、严重程度评分及RNS异常可能影响妊娠期MG临床症状,MG病情的加重多见于妊娠早晚期。新生儿一过性肌无力症状并不常见,但须神经科和妇产科医师严密监测。“,”Objective:To study the clinical characteristics of pregnant patients with myasthenia gravis (MG) and the influence of MG to pregnancy.Methods:A retrospective study was conducted including 28 MG patients with 38 pregnancies admitted to the 8th Medical Center of PLA General Hospital between January 2013 and October 2018. Data were collected including clinical scores of MG, serum level of acetylcholine receptor (AChR) antibodies, abnormal repetitive nerve stimulation (RNS) and history of thymectomy before pregnancy. The course of pregnancy, delivery and neonatal outcome were also analyzed. According the outcome of MG, patients were divided into three groups, i.e. improvement group, stable group and deterioration group.Results:(1) The age of MG patients ranged from 21 to 36 (27±4) years. The previous course of MG was 0.5-17.2 (7.4±5.8)years. Based on Osserman clinical type, type ⅡA was the most common one [44.1% (15/34)], followed with type Ⅰ [29.4% (10/34)], type ⅡB [23.5% (8/34)] and type Ⅳ (2.9%).(2)There were 38 pregnancies in 28 women whose pregnancy outcomes resulted in one spontaneous abortion, three embryonic arrest and 34 live births. All abortions developed in the first trimester. Among the 34 pregnancies with live births, the symptoms of MG improved in 16 pregnancies (47.1%), whereas those deteriorated in 10 pregnancies (29.4%) during the first or third trimester and remained stable in 8 pregnancies (23.5%). (3) Compared with improvement group and stable group, the deterioration group had shorter duration of MG [(1.1±0.5) years vs. (7.1±5.1) years, (9.0±5.4) years respectively], higher clinical scores (20.9±6.0 vs. 14.8±6.6,13.3±5.0) and more frequent abnormal RNS(9/10 vs. 8/16, 4/8) and type ⅡB(6/10n vs. 1/16, 1/8) before pregnancy. Positive rate of serum AChR antibody and percentage of thymectomy before pregnancy were comparable between three groups. (4) Spinal anesthesia was performed in 23 pregnancies and 11 cases were vaginal delivery. No transient neonatal MG were found in live-born infants.n Conclusions:Pregnancy in patients with under-controlled myasthenia gravis should not be discouraged. The outcome of MG is affected by the duration of MG, MG score and RNS before pregnancy. The first and third trimesters of pregnancy are considered high-risk periods for MG exacerbations. Neonatal transient myasthenia is uncommon, but the newborn should be carefully monitored by obstetricians and neurologists.
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