妊娠期肢腰肌营养不良症:叙述性综述

IF 2.1 3区 医学 Q2 OBSTETRICS & GYNECOLOGY Archives of Gynecology and Obstetrics Pub Date : 2024-09-16 DOI:10.1007/s00404-024-07738-1
H. Shafeeq Ahmed
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引用次数: 0

摘要

肢腰肌营养不良症(LGMD)给孕期妇女带来了独特的挑战,需要全面的护理和量身定制的管理策略。本综述旨在探讨妊娠期 LGMD 患者所面临的独特挑战和所需的管理策略。目前已确定的遗传亚型超过 30 种,而且通过先进的诊断技术还有可能发现更多亚型,因此孕前咨询在告知未来父母生育风险和可用选择方面起着至关重要的作用。包括心脏和肺部评估在内的基线评估对于指导产前护理以及基因检测以进行精确诊断和咨询至关重要。通过呼吸运动、心脏监测以及个性化的运动和营养计划来优化孕产妇健康,对于避免潜在并发症至关重要。在怀孕期间,产科医生和专科医生之间的合作护理对密切监测母体和胎儿的健康状况非常重要。考虑到肌肉力量、骨盆大小和胎儿外形等因素,个性化的分娩方式至关重要。虽然阴道分娩已被证明是可行的,但应始终牢记紧急剖宫产的必要性。区域麻醉是首选,并对潜在的呼吸支持进行积极规划。布比卡因已被证明对硬膜外导管有效,可使用吗啡和芬太尼等阿片类药物延长缓解时间,同时还要评估患者的呼吸功能。产后注意事项包括疼痛管理、行动支持、母乳喂养协助和情感支持。早期活动和量身定制的物理治疗方案可促进最佳恢复,同时需要全面的母乳喂养指导,以应对与肌肉无力有关的挑战。获得心理健康资源和支持网络对于帮助患者在控制 LGMD 的同时应对为人父母的情感需求至关重要。通过满足妊娠期 LGMD 患者的独特需求,医疗服务提供者可以优化孕产妇和胎儿的预后,同时在他们为人父母的过程中提供支持。
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Limb-girdle muscular dystrophy in pregnancy: a narrative review

Limb-girdle muscular dystrophy (LGMD) poses unique challenges for women during pregnancy, necessitating comprehensive care and tailored management strategies. The present narrative review aims to examine the unique challenges and management strategies required for women with LGMD during pregnancy. With over 30 genetic subtypes identified and the potential for additional discoveries through advanced diagnostic techniques, preconception counseling plays a crucial role in informing prospective parents about reproductive risks and available options. Baseline assessments, including cardiac and pulmonary evaluations, are essential to guide antenatal care, alongside genetic testing for precise diagnosis and counseling. Optimizing maternal health through respiratory exercises, cardiac monitoring, and individualized exercise and nutrition plans is paramount to avoid potential complications. During pregnancy, close monitoring of maternal and fetal well-being is important, with collaborative care between obstetricians and specialists. An individualized approach to delivery mode considering factors such as muscle strength, pelvic size, and fetal presentation is crucial. While vaginal delivery has been proven to be possible, the need for an emergency cesarean delivery should always be kept in mind. Regional anesthesia is preferred, with proactive planning for potential respiratory support. Bupivacaine has been shown to be effective with epidural catheters that may be used for prolonged relief with opioids like morphine and fentanyl, while also evaluating the patients’ respiratory function. Postpartum considerations include pain management, mobility support, breastfeeding assistance, and emotional support. Early mobilization and tailored physiotherapy regimens may promote optimal recovery, while comprehensive breastfeeding guidance is needed to address challenges related to muscle weakness. Access to mental health resources and support networks is essential to helping individuals cope with the emotional demands of parenthood alongside managing LGMD. By addressing the unique needs of pregnant individuals with LGMD, healthcare providers can optimize maternal and fetal outcomes while supporting individuals in their journey to parenthood.

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来源期刊
CiteScore
4.70
自引率
15.40%
发文量
493
审稿时长
1 months
期刊介绍: Founded in 1870 as "Archiv für Gynaekologie", Archives of Gynecology and Obstetrics has a long and outstanding tradition. Since 1922 the journal has been the Organ of the Deutsche Gesellschaft für Gynäkologie und Geburtshilfe. "The Archives of Gynecology and Obstetrics" is circulated in over 40 countries world wide and is indexed in "PubMed/Medline" and "Science Citation Index Expanded/Journal Citation Report". The journal publishes invited and submitted reviews; peer-reviewed original articles about clinical topics and basic research as well as news and views and guidelines and position statements from all sub-specialties in gynecology and obstetrics.
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