Pregnancy in women with congenital physical disabilities: Risk factors, birth outcomes, and mediation

在过去，大多数先天性畸形患者的平均寿命都有了很大提高 几十年，使大多数人能够在生殖年龄存活下来。因为一个物理的存在 残疾通常不会影响生育能力或对家庭的渴望，重要的是要了解 受影响的妇女怀孕。有证据表明，对残疾有不同定义的妇女， 可能会有出生体重过轻或早产的婴儿。有限的研究表明， 这些分娩结果的相关风险因素在孕妇中更常见， 残疾人与普通人相比。例如，吸烟、饮酒和肥胖 增加不良后果的风险，并且似乎在有以下情况的孕妇中不成比例地高 残疾。然而，迄今为止还没有研究研究探讨妊娠暴露与不良分娩的关系 在身体残疾妇女中取得的成果。在育龄妇女中， 通常源于先天性肌肉和神经肌肉缺陷（cMND），定义为脊柱裂，脑 瘫痪、肌肉萎缩症和肢体异常（挛缩、缺失或缩短）。行动不便 和灵活性会影响日常生活活动，并导致慢性疼痛，睡眠障碍， 心理问题治疗这些症状所需的药物，以及其他行为应对 机制，可能与出生结果有关，进一步提高了研究妊娠过程的必要性。 和生育结果之间的cMND影响的妇女。本研究将利用丰富的数据资源-- 波士顿大学斯隆流行病学中心出生缺陷研究。从1976年到2015年， 出生时有或没有先天性异常的婴儿被问及有关一系列事件的详细问题， 和怀孕期间的暴露，以确定出生缺陷的风险因素。妇女 特别询问他们是否天生患有脊柱裂或任何大脑，头部，脊柱，脊髓异常， 肌肉、骨骼、手臂或腿部。这项研究将比较132名妇女谁被确定为有一个 对528名未受影响的妇女进行以下妊娠暴露的cMND：吸烟、饮酒、产前检查、 怀孕体重指数，维生素补充，肾脏/膀胱感染，止痛药，睡眠 药物和精神药物。常见不良出生结果的比较将包括 早产低出生体重和巨大儿此外，调解分析将衡量 cMND和出生结果之间的关联是通过妊娠暴露介导的。一个 据估计，212，000名美国育龄妇女患有cMND，对她们来说，怀孕可能需要特殊的治疗。 服务有必要了解先天性心脏病女性的妊娠过程和结局 身体残疾作为确保最佳护理和风险预防的一个步骤。该项目利用 一个独特的数据资源，以提供有价值的信息，为此目的。

The average lifespan of individuals born with most congenital malformations has vastly improved in the past decades, allowing the majority to survive through the reproductive years. Because the presence of a physical disability typically does not affect fertility or the desire for a family, it is important to understand the course of pregnancy among affected women. Evidence shows that women with varying definitions of disability are more likely to have babies born with low birth weights or prematurely. The limited research available suggests that relevant risk factors for these birth outcomes tend to be more common in pregnant women with varying disabilities compare to the general population. For example, cigarette smoking, alcohol drinking, and obesity increase risks of adverse outcomes and appear to be disproportionately higher in pregnant women with disabilities. However no study to date has examined pregnancy exposures in relation to adverse birth outcomes among women with physical disabilities. In women of childbearing ages, physical disabilities most often stem from congenital muscular and neuromuscular defects (cMND), defined as spina bifida, cerebral palsy, muscular dystrophy, and limb anomalies (contractures, missing or shortened). Impairments in mobility and dexterity can impact activities of daily living and result in chronic pain, sleep disturbances, and psychosocial problems. Medications needed for the treatment of these symptoms, and other behavioral coping mechanisms, may be associated with birth outcomes, raising further the need to study the course of pregnancy and birth outcomes among cMND-affected women. This study will take advantage of rich data resource - the Boston University Slone Epidemiology Center Birth Defects Study. From 1976 to 2015, over 68,000 mothers of babies born with and without congenital anomalies were asked detailed questions about a range of events and exposures during pregnancy for the purpose of identifying risk factors for birth defects. Women were specifically asked if they were born with spina bifida or any anomalies of the brain, head, spine, spinal cord, muscles, bones, arms, or legs. This study will compare the 132 women who were identified as having a cMND to 528 unaffected women for the following pregnancy exposures: cigarette smoking, alcohol intake, pre- pregnancy body mass index, vitamin supplementation, kidney/bladder infection, pain medications, sleep medications, and psychoactive medications. Comparisons of common adverse birth outcomes will include premature birth, low birth weight, and macrosomia. In addition, mediation analyses will measure the amount of an association between cMND and birth outcomes that is mediated through pregnancy exposures. An estimated 212,000 US women of childbearing ages have a cMND, for whom pregnancy may require special services. It is necessary to understand the course of pregnancy and outcomes among women with congenital physical disabilities as a step toward ensuring optimal care and risk prevention. This project takes advantage of a unique data resource to provide valuable information for this purpose.