PDF(Pubmed)
Abstract：
OBJECTIVE: To co-create expert guidelines for the management of pregnancy, birth, and postpartum recovery in the context of hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD).
METHODS: Scoping Review and Expert Co-creation.
METHODS: United Kingdom, United States of America, Canada, France, Sweden, Luxembourg, Germany, Italy, and the Netherlands.
METHODS: Co-creators (n = 15) included expertise from patients and clinicians from the International Consortium on the Ehlers-Danlos syndromes and Hypermobility Spectrum Disorders, facilitated by the Ehlers-Danlos Society.
METHODS: A scoping review using Embase, Medline, the Cochrane Central Register of Controlled Trials and CINHAL was conducted from May 2022 to September 2023. Articles were included if they reported primary research findings in relation to childbearing with hEDS/HSD, including case reports. No language limitations were placed on our search, and our team had the ability to translate and screen articles retrieved in English, French, Spanish, Italian, Russian, Swedish, Norwegian, Dutch, Danish, German, and Portuguese. The Mixed Methods Appraisal Tool was used to assess bias and quality appraise articles selected. The co-creation of guidelines was based on descriptive evidence synthesis along with practical and clinical experience supported by patient and public involvement activities.
RESULTS: Primary research studies (n = 14) and case studies (n = 21) including a total of 1,260,317 participants informed the co-creation of guidelines in four overarching categories: 1) Preconceptual: conception and screening, 2) Antenatal: risk assessment, management of miscarriage and termination of pregnancy, gastrointestinal issues and mobility, 3) Intrapartum: risk assessment, birth choices (mode of birth and intended place of birth), mobility in labor and anesthesia, and 4) Postpartum: wound healing, pelvic health, care of the newborn and infant feeding. Guidelines were also included in relation to pain management, mental health, nutrition and the common co-morbidities of postural orthostatic tachycardia syndrome, other forms of dysautonomia, and mast cell diseases.
CONCLUSIONS: There is limited high quality evidence available. Individualized strategies are proposed for the management of childbearing people with hEDS/HSD throughout pregnancy, birth, and the postpartum period. A multidisciplinary approach is advised to address frequently seen issues in this population such as tissue fragility, joint hypermobility, and pain, as well as common comorbidities, including dysautonomia and mast cell diseases.
METHODS: Scoping Review and Expert Co-creation.
METHODS: United Kingdom, United States of America, Canada, France, Sweden, Luxembourg, Germany, Italy, and the Netherlands.
METHODS: Co-creators (n = 15) included expertise from patients and clinicians from the International Consortium on the Ehlers-Danlos syndromes and Hypermobility Spectrum Disorders, facilitated by the Ehlers-Danlos Society.
METHODS: A scoping review using Embase, Medline, the Cochrane Central Register of Controlled Trials and CINHAL was conducted from May 2022 to September 2023. Articles were included if they reported primary research findings in relation to childbearing with hEDS/HSD, including case reports. No language limitations were placed on our search, and our team had the ability to translate and screen articles retrieved in English, French, Spanish, Italian, Russian, Swedish, Norwegian, Dutch, Danish, German, and Portuguese. The Mixed Methods Appraisal Tool was used to assess bias and quality appraise articles selected. The co-creation of guidelines was based on descriptive evidence synthesis along with practical and clinical experience supported by patient and public involvement activities.
RESULTS: Primary research studies (n = 14) and case studies (n = 21) including a total of 1,260,317 participants informed the co-creation of guidelines in four overarching categories: 1) Preconceptual: conception and screening, 2) Antenatal: risk assessment, management of miscarriage and termination of pregnancy, gastrointestinal issues and mobility, 3) Intrapartum: risk assessment, birth choices (mode of birth and intended place of birth), mobility in labor and anesthesia, and 4) Postpartum: wound healing, pelvic health, care of the newborn and infant feeding. Guidelines were also included in relation to pain management, mental health, nutrition and the common co-morbidities of postural orthostatic tachycardia syndrome, other forms of dysautonomia, and mast cell diseases.
CONCLUSIONS: There is limited high quality evidence available. Individualized strategies are proposed for the management of childbearing people with hEDS/HSD throughout pregnancy, birth, and the postpartum period. A multidisciplinary approach is advised to address frequently seen issues in this population such as tissue fragility, joint hypermobility, and pain, as well as common comorbidities, including dysautonomia and mast cell diseases.
摘要：
目的：共同制定妊娠管理专家指南，出生,在高流动性Ehlers-Danlos综合征（hEDS）和高流动性谱系障碍（HSD）的背景下，以及产后恢复。
方法：范围审查和专家共同创造。
方法：英国，美利坚合众国,加拿大,法国,瑞典,卢森堡,德国,意大利,和荷兰。
方法：共同创造者（n=15）包括来自国际协会的患者和临床医生在Ehlers-Danlos综合征和高流动性频谱障碍方面的专业知识，由Ehlers-Danlos协会协助。
方法：使用Embase进行范围审查，Medline,Cochrane中央对照试验注册和CINHAL于2022年5月至2023年9月进行。如果他们报告了与hEDS/HSD生育有关的主要研究结果，包括病例报告。我们的搜索没有语言限制，我们的团队有能力翻译和筛选用英语检索的文章，法语,西班牙语,意大利语,俄语,瑞典语,挪威语,荷兰人,丹麦语,德语,和葡萄牙语。使用混合方法评估工具对所选文章进行偏倚和质量评估。共同创建指南是基于描述性证据综合以及患者和公众参与活动支持的实践和临床经验。
结果：主要研究研究（n=14）和案例研究（n=21），包括总共1,260,317名参与者在四个总体类别中共同制定了指南：1）概念：概念和筛查，2)产前：风险评估，流产和终止妊娠的管理，肠胃问题和活动能力,3）产时：风险评估，出生选择（出生方式和预期出生地点），分娩和麻醉的流动性，和4）产后：伤口愈合，骨盆健康，照顾新生儿和婴儿喂养。有关疼痛管理的指南也包括在内，心理健康,营养和体位性心动过速综合征的常见合并症，其他形式的自主神经失调,和肥大细胞疾病。
结论：可获得的高质量证据有限。提出了个性化策略，用于在整个怀孕期间管理hEDS/HSD的育龄患者，出生,和产后。建议采用多学科方法来解决该人群中经常出现的问题，例如组织脆性，关节过度活动,和痛苦，以及常见的合并症，包括自主神经失调和肥大细胞疾病.
方法：范围审查和专家共同创造。
方法：英国，美利坚合众国,加拿大,法国,瑞典,卢森堡,德国,意大利,和荷兰。
方法：共同创造者（n=15）包括来自国际协会的患者和临床医生在Ehlers-Danlos综合征和高流动性频谱障碍方面的专业知识，由Ehlers-Danlos协会协助。
方法：使用Embase进行范围审查，Medline,Cochrane中央对照试验注册和CINHAL于2022年5月至2023年9月进行。如果他们报告了与hEDS/HSD生育有关的主要研究结果，包括病例报告。我们的搜索没有语言限制，我们的团队有能力翻译和筛选用英语检索的文章，法语,西班牙语,意大利语,俄语,瑞典语,挪威语,荷兰人,丹麦语,德语,和葡萄牙语。使用混合方法评估工具对所选文章进行偏倚和质量评估。共同创建指南是基于描述性证据综合以及患者和公众参与活动支持的实践和临床经验。
结果：主要研究研究（n=14）和案例研究（n=21），包括总共1,260,317名参与者在四个总体类别中共同制定了指南：1）概念：概念和筛查，2)产前：风险评估，流产和终止妊娠的管理，肠胃问题和活动能力,3）产时：风险评估，出生选择（出生方式和预期出生地点），分娩和麻醉的流动性，和4）产后：伤口愈合，骨盆健康，照顾新生儿和婴儿喂养。有关疼痛管理的指南也包括在内，心理健康,营养和体位性心动过速综合征的常见合并症，其他形式的自主神经失调,和肥大细胞疾病。
结论：可获得的高质量证据有限。提出了个性化策略，用于在整个怀孕期间管理hEDS/HSD的育龄患者，出生,和产后。建议采用多学科方法来解决该人群中经常出现的问题，例如组织脆性，关节过度活动,和痛苦，以及常见的合并症，包括自主神经失调和肥大细胞疾病.
