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82 例意大利患者的关节过度活动综合征(也称埃勒斯-当洛斯综合征 Ⅰ型)的妇产科影响。

Gynecologic and obstetric implications of the joint hypermobility syndrome (a.k.a. Ehlers-Danlos syndrome hypermobility type) in 82 Italian patients.

机构信息

Division of Medical Genetics, Department of Molecular Medicine, Sapienza University, San Camillo-Forlanini Hospital, Rome, Italy.

出版信息

Am J Med Genet A. 2012 Sep;158A(9):2176-82. doi: 10.1002/ajmg.a.35506. Epub 2012 Jul 27.

Abstract

Joint hypermobility syndrome (JHS) emerges as likely the most common clinical form of Ehlers-Danlos syndrome. Given the striking predominance of affected women, practitioners often face gynecologic and obstetric issues. However, their decisions are still based on personal experience rather than literature due to the lack of a consistent body of evidence. We collected a set of gynecologic and obstetric features in 82 post-puberal women with JHS attending two Italian centers. Common gynecologic findings were dysmenorrhea (82.9%), meno/metrorrhagias (53.7%), irregular menses (46.3%), and dispareunia/vulvodinia (31.7%). Forty women were nulliparous and 42 had one or more pregnancy for a total of 93 diagnosed conceptions. Of them, 16.1% were spontaneous abortions, 6.5% voluntary interruptions, 10.7% preterm deliveries, and 66.7% deliveries at term. Overall outcome of proceeding pregnancies was good with no stillbirth and fetal/neonatal hypoxic/ischemic event. Non-operative vaginal delivery was registered in 72.2%, forceps/vacuum use in 5.5% and cesarean in 22.3%. Local/total anesthesia was successfully performed in 17 pregnancies without any problem. Major post-partum complications included abnormal scar formation after cesarean or episiotomy (46.1%), hemorrhage (19.4%), pelvic prolapses (15.3%), deep venous thrombosis (4.2%), and coccyx dislocation (1.4%). Prolapses were the most clinically relevant complication and associated with episiotomy. Gathered data were discussed for practically oriented considerations.

摘要

关节过度活动综合征(JHS)可能是埃勒斯-当洛斯综合征最常见的临床形式。鉴于受影响的女性数量显著居多,医生经常会遇到妇科和产科问题。然而,由于缺乏一致的证据体系,他们的决策仍然基于个人经验,而不是文献。我们收集了意大利两个中心的 82 名青春期后 JHS 女性的一组妇科和产科特征。常见的妇科发现包括痛经(82.9%)、绝经/月经过多(53.7%)、月经不规律(46.3%)和性交困难/外阴痛(31.7%)。40 名患者为未婚,42 名患者有一次或多次妊娠,总计诊断为 93 次妊娠。其中,16.1%为自然流产,6.5%为自愿终止妊娠,10.7%为早产,66.7%为足月分娩。所有后续妊娠的结局均良好,无死胎和胎儿/新生儿缺氧/缺血事件。72.2%为非手术阴道分娩,5.5%为产钳/真空吸引,22.3%为剖宫产。17 例局部/全身麻醉成功,无任何问题。主要的产后并发症包括剖宫产或会阴切开术后异常瘢痕形成(46.1%)、出血(19.4%)、骨盆脱垂(15.3%)、深静脉血栓形成(4.2%)和尾骨脱位(1.4%)。脱垂是最具临床相关性的并发症,与会阴切开术有关。收集的数据进行了讨论,以提供实用的考虑。

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