https://orcid.org/0000-0001-5767-0708
Figures
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).
Setting
United Kingdom, United States of America, Canada, France, Sweden, Luxembourg, Germany, Italy, and the Netherlands.
Sample
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.
Citation: Pezaro S, Brock I, Buckley M, Callaway S, Demirdas S, Hakim A, et al. (2024) Management of childbearing with hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders: A scoping review and expert co-creation of evidence-based clinical guidelines. PLoS ONE 19(5): e0302401. https://doi.org/10.1371/journal.pone.0302401
Editor: Martin E. Matsumura, Geisinger Health System, UNITED STATES
Received: January 11, 2024; Accepted: April 2, 2024; Published: May 15, 2024
Copyright: © 2024 Pezaro et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Data Availability: All relevant data are within the paper and its Supporting information files.
Funding: The author(s) received no specific funding for this work.
Competing interests: SP reports receiving honorariums from the Ehlers-Danlos Society. AH reports receiving honorariums from the Ehlers-Danlos Society. NB reports receiving honorariums from the Ehlers-Danlos Society. Other authors report no conflict of interest.
Introduction
The Ehlers-Danlos syndromes (EDS) are a group of underdiagnosed, heritable connective tissue disorders characterized by generalized joint hypermobility (GJH), skin hyperextensibility and tissue fragility. Thirteen types of EDS were identified by the International Consortium on the Ehlers-Danlos Syndromes in 2017 [1], and a fourteenth subtype has since been identified [2]. Hypermobile EDS (hEDS) is the most common type of EDS, mainly symptomatic in people assigned female at birth [3,4]. Updated diagnostic criteria for hEDS released in 2017 narrowly define hEDS based on the presence of GJH, multiple features of underlying connective tissue weakness, with or without confirmed family history of the same diagnosis [1]. Those presenting with GJH, musculoskeletal complications and pain with or without associated comorbidities, who do not fulfill criteria of hEDS may fall under the diagnostic category of hypermobility spectrum disorders (HSD), for which the same management approaches will apply [5,6]. Thus hEDS/HSD are referred to together throughout this article, though we recognize that more will be diagnosed with HSD than hEDS. hEDS/HSD is likely underdiagnosed overall but recent estimations suggest that the combined prevalence of hEDS and HSD in the order of 1 in 600 to 1 in 900 [4], with many pregnancies affected [7]. Separate prevalence estimates for hEDS and HSD are not currently available. Patients with hEDS/HSD present with multisystem signs, symptoms, and comorbidities the most common of which are outlined in Table 1, adapted from Hamonet and colleagues with permission [8].
Regarding frequencies presented in Table 1, the control group had symptoms at <10% frequency, with the following exceptions: sleeping problems (26%), upper respiratory infections (23%), bloating or distension caused by gas (19%), gastroesophageal reflux (13%), and meno/metrorrhagia (11%).
Prevalence of obstetric, pelvic and reproductive system issues reported by those childbearing with hEDS/HSD are significantly greater than in the general childbearing population [9,10]. Symptoms can be debilitating; they may either be exacerbated or improve during pregnancy and birth, when hormonal levels are elevated [7,11–13]. Still, there is scarce literature related to perinatal care in the context of hEDS/HSD, leading to potential misconceptions and a lack of knowledge in some health care professionals [7,14]. Discussions with patient groups and members of the International Consortium on the Ehlers-Danlos syndromes and Hypermobility Spectrum Disorders established the importance of and need to have international expert guidelines drawing from existing evidence and expert opinion to provide guidance to care providers and persons with these conditions in childbearing. Moreover, evidence-based guidelines have been most commonly requested by both perinatal professionals and those childbearing with hEDS/HSD in a large international survey investigating perinatal staff’s knowledge and confidence in supporting people with hEDS/HSD, and people with hEDS/HSD’s experiences of perinatal care [14].
Considering the above, the overarching aim of this research was to co-create international evidence-based expert guidelines on the management of pregnancy, birth, and post-natal recovery in the context of hEDS/HSD. A scoping review was conducted to inform the co-creation of these guidelines and answer the following question: What is known from the literature about pregnancy, birth, and post-natal recovery in people with hEDS/HSD?
Scoping reviews differ from systematic reviews and lend themselves to scoping a body of literature while utilising the experience and expertise of their authors [15]. They are also complementary to co-creating guidelines and guidelines such as these [16] and are recommended to addresses several questions from a diverse body of literature pertaining to a broad topic. As such, a scoping review was considered the most rigorous approach to evaluate the evidence and inform these guidelines, rather than a systematic review, which is methodologically suited to address only one question. Our scoping review followed a preferred framework [17], consisting of the following steps: (1) identifying the research question(s), (2) identifying relevant studies, (3) study selection, (4) charting the data, (5) collating, summarising, and reporting the results, and (6) optional consultation exercises. We also included patient representatives and ensured stakeholder engagement throughout to enhance the rigor of our scoping approach [18]. This also met our objective with regards to using the findings of a scoping review to inform the co-creation of evidence-based guidelines in this context.
Methods
This research was undertaken using evidence-based approaches [19]. Alongside a scoping review, the three Co’s framework of ‘Co-define’ (examining problems and positive aspects), ‘Co-design’ (prioritising problems and designing solutions), ‘Co-refine’ (co-produce and refine together) was used to co-create the guidance presented [20]. Such co-creation is underpinned by participatory action research [21–23] and design thinking [24,25]. In person consensus meetings along with Patient and Public Involvement (PPI) activities involving wider patient and public groups and convened by the Ehlers-Danlos Society were also undertaken in finalizing the guidelines presented.
Co-creator recruitment began after ethical approval was granted via the lead author’s University in March 2022 (Project P135062). Our sampling strategy was purposive. Members of the International Consortium on the Ehlers-Danlos syndromes and Hypermobility Spectrum Disorders (www.ehlers-danlos.com/international-consortium), including those with lived experience of both hEDS/HSD and childbearing, along with clinicians and academics were sent participant information via email and were invited to participate.
Once informed consent was secured, each co-creator was given access to the project’s co-creation space online and invited to visit the ‘co-creating welfare’ project website (http://ccw.southdenmark.eu/) to become familiar with the principles of co-creation. Those who gave their consent to participate were invited to engage in a series of online co-creation workshops hosted by the Ehlers-Danlos Society and led by the principal author.
During the first co-creation workshop, activities focused on ‘co-defining’ what the issues, problems, and positive aspects of perinatal care in this context are. Thereafter, co-creators were invited to prioritise the problems identified, find solutions, and ‘co-design’ expert guidance together in real time. These were then co-produced and refined together. Both during and outside of bi-weekly ‘co-refining’ workshops, co-creators were invited to ‘co-refine’ guidelines via an iterative succession of discussions and annotated co-refinements.
Co-creation began in March 2022 and concluded in November 2023 following a face-to-face consensus meeting held in Arizona (August 2022), and public and patient involvement activities held in Rome (September 2022) hosted by the Ehlers-Danlos Society. During 2023, four further co-refining workshops were hosted to discuss residual deviating views and ideas until unanimity was reached. In line with our evidence-based approach [19], co-creation activities were supported by our scoping review, which was last updated in September 2023. Expert co-creators consulted the findings of each article included and used these, along with their practical knowledge and/or experiences to support and inform the co-created guidelines.
The search strategy for our scoping review is outlined in Table 2 and was formulated in partnership with librarians at the Erasmus MC Medical Library in Rotterdam in the Netherlands, inclusive of previous nomenclature. The PRISMA extension for scoping reviews (PRISMA-ScR) was used to guide reporting (See checklist in S1 File).
Screening of the articles was led by two members of the team (SP and ILR) who engaged all co-creators in decision making during bi-weekly meetings. Final articles were included to inform the guidelines if they reported primary research findings in relation to childbearing with hEDS/HSD. Case reports were also included to inform the guidelines presented. No limits were placed with regards to the language of articles to be screened, and our team had the ability to translate and screen articles retrieved in the following languages: English, French, Spanish, Italian, Russian, Swedish, Norwegian, Dutch, Danish, German, and Portuguese. Articles were excluded if they did not relate to childbearing in the context of hEDS/HSD. Alongside the inclusion of newly published peer reviewed articles shared via professional networks, the reference lists of identified articles and literature reviews were also screened for additional relevant citations. Reviews of the literature were excluded if they did not also feature a clinical case study. Authors of all selected articles were invited to offer any further available evidence for inclusion. To further enhance the rigor of our approach, primary research articles (excluding case studies) which met inclusion criteria were then quality appraised using the Mixed Methods Appraisal Tool (MMAT), which enables researchers to simultaneously evaluate the validity and reliability of both quantitative and qualitative empirical studies [26]. Quality scores range from * if one criterion is met to ***** if all five criteria are met [27]. Final appraisal scores were proposed in consultation by 3 members of the team (ILR, SD and SP), and then agreed by the wider co-creation team.
Co-creators considered the identified evidence and contextualized it alongside the co-production of guidelines while engaging the principles of the WHO-INTEGRATE evidence to decision framework [28]. In line with best practice and where evidence was lacking [19], co-creators formulated guidelines based on practical and clinical experience, and with input from those with lived experiences. Once guidelines were finalized, external members of the International Consortium with relevant subject expertise distinct from those within the co-creation team were invited to assess the final guidelines for clarity and relevance.
This research occurred in a context where minoritized Black and other ethnic communities, the Lesbian, Gay, Bisexual, Transgender, Queer and/or Questioning, Intersex, Asexual, Two-Spirit (LGTBQIA2S+) communities and those with low socioeconomic status face challenges and experience discrimination in healthcare every day [29]. Consequently, gender-inclusive and person-first language was used throughout reporting. All co-creators were unanimous in approving the finalized guidelines reported here.
Patient and public involvement activities
Patient and stakeholder engagement in guideline development is internationally advocated [30]. We report the Patient and Public Involvement (PPI) activities conducted in line with the Guidance for Reporting Involvement of Patients and the Public (GRIPP2) short form checklist [31]. To reduce the potential for bias, prior relationships with those engaging in PPI activities and the co-creation team were minimal, and activities remained congruent with best practice [30].
Aim.
The aim of PPI in this study was to involve members of the public and those with lived experience of hEDS/HSD and childbearing in the co-creation of international expert guidelines for the management of pregnancy, birth, and post-natal recovery in the context of hEDS/HSD. We were also keen to co-author and disseminate this publication with those who have had experience of childbearing with hEDS/HSD.
Methods.
Patient representatives were involved in this project from its conception, both as co-creators, authors, and consultants with lived experience of hEDS/HSD and/or childbirth. All patients and public attending conferences hosted by the Ehlers-Danlos Society in both Arizona (August 2022) and Rome (September 2022) were invited to comment upon and shape guidelines in partnership with the co-creation team in person. Further comments were also solicited via the Ehlers-Danlos Society’s Global Affiliation Program newsletter, and via a separate EDS affiliate focus group facilitated online by MB including 12 participants representing USA, Canada, Sweden, Luxembourg, and Germany. All comments resulting from these activities were collated together and shared with the co-creation team. They were then examined collectively to identify common topics, ideas, and patterns of meaning which were then grouped into themes.
Results.
Themes related to ‘key topics’ for inclusion in the guidelines and related to the management of miscarriage and termination of pregnancy, comorbidities, causal relationships, anesthesia, incontinence, and risk assessments along with the management of symptoms such as enhanced joint laxity and pain. Those who engaged in PPI requested guidelines from the co-creation team with regards to the preconceptual, antenatal, intrapartum, and postpartum periods.
Discussion and conclusions.
PPI influenced this study substantially overall. A distinctly positive aspect of engaging in PPI from the start meant that we could stay focussed on what mattered most and communicate to professionals using the voices of those with lived experience. Nevertheless, it was challenging to balance the knowledge, voices, and concerns of experts with lived experience and practicing clinicians.
Reflections.
Due to previous negative experiences with clinicians in healthcare, some engaged with PPI activities were doubtful as to the ability of clinicians to use their initiative or provide basic, competent, and compassionate care, and thus requested that guidelines included basic principles of healthcare practice. This was frustrating to clinical professionals, who expressed fears that clinicians would disengage from guidelines which repeated their basic training. The rebuilding of trust between clinicians and those with lived experience in this area may remove this as a barrier to future PPI activities.
Results
A total of 15 co-creators joined in meeting the aim of this research from the United Kingdom, United States of America, Canada, France, and the Netherlands. Co-creators represented a variety of professions including midwifery, obstetrics, maternal fetal medicine, rheumatology, registered dietitians, and nutritionists, physical therapy, clinical geneticists, nursing, clinical research, and pain management. Co-creators also included patient advocates with lived experience of both hEDS/HSD and childbearing. All co-creators joined in authoring the final report and guidelines, and thus are listed as co-authors to this article.
Fig 1 outlines how the final articles (n = 35) resulting from our literature searches were identified for inclusion following the removal of duplicates.
The final articles which met our inclusion criteria consisted of primary research studies (n = 14) and case studies (n = 21). They included a total of 1,260,317 participants. Our collation and summarising of results are reported in Table 3, which presents article details, findings, and quality appraisal scores.
Final expert guidelines were broadly grouped into the following categories presented in Table 4: Preconceptual (conception and screening), Antenatal (risk assessment, management of miscarriage and termination of pregnancy and mobility), Intrapartum (risk assessment, birth choices, mode of birth/intended place of birth, mobility in labour and anaesthesia), and Postpartum (wound healing, pelvic health, care of the newborn and infant feeding). Our supplement also outlines guidance in relation to physical therapy (see table in S2 File). These are intended to act as guides rather than policy in the understanding that professionals take an individualized approach to care.
Discussion
This article presents an international consensus of expert guidelines in the management of childbearing with hEDS/HSD brought about using systematic and co-creation approaches along with a variety of PPI activities. While we have built upon previous care considerations [11–13], these guidelines represent the first of their kind from an international multidisciplinary collaboration, including patient perspectives. In our experts’ view, many of the guidelines made can be applicable in other subtypes of EDS (except vascular EDS), though each type should be considered individually via future research. Practitioners may also consider these expert guidelines for those with generalized HSD, as underlying care principles will be largely the same. Moreover, as JHS is indistinguishable from hEDS/HSD and considered likely allelic, this guideline should also apply to those diagnostically labelled as having JHS. We also offer strategies in relation to managing the comorbidities associated with hEDS/HSD, although we recognize that further research is required in these areas, and more detailed expert consensus’ remains warranted [73,74,123].
Guidelines were co-created into the following categories: Preconceptual (conception and screening), Antenatal (risk assessment, management of miscarriage and termination of pregnancy and mobility), Intrapartum (risk assessment, birth choices, mode of birth/intended place of birth, mobility in labor and anesthesia), and Postpartum (wound healing, pelvic health, care of the newborn and infant feeding). Our use of the wider literature in co-creation adds to earlier syntheses and understandings in this area [124,125]. Yet further research is required with regards to obstetric outcomes dependent upon the pregnant person’s and the unborn’ EDS status, particularly where outcomes in relation to specific types of EDS are not reported separately [82,126].
Due to their inextricable links, PPI activities and co-creators also directed us to provide expert guidelines in relation to some of the comorbidities of hEDS/HSD such as mast cell diseases and spinal instability [127]. Equally, these guidelines evolved to encompass dysautonomia, orthostatic intolerance with or without orthostatic hypotension and POTS, of which between 70% to ≥80% of those with hEDS/HSD may also experience symptoms related to childbearing [127]. Guidelines were also included in relation to pain management, mental health, nutrition, supplementation, allergies with immune responses, and overall wellbeing. Further resources and citations are also provided and should be referred to, particularly in relation to paediatrics [116].
In the assessment of risk in planning both the mode and place of birth, the anticipation of increased blood loss will be key in all cases. Individuals with hEDS/HSD typically have fragile capillaries and tissue, predisposing them to bruising and hematomas. In addition, some will have an abnormal interaction between Von Willebrand factor, platelets, and collagen resulting in suboptimal blood clotting which in turn can lead to heavier and prolonged bleeding [128]. During pregnancy, increased mucosal fragility can result in spontaneous bleeding (e.g., epistaxis and gingival). There may similarly be increased bleeding during childbirth [10,34,40,41]. Yet some large retrospective studies have found no increased risk for bleeding in such cases [35,36]. Nevertheless, alongside other more well-known treatments, we have been able to endorse a wider variety of medicines such as Desmopressin and Tranexamic Acid to control bleeding where applicable [99,100]. Due to the potential concern for precipitous birth in this population [7,10], it may be prudent to plan for births in a community setting with attention to distance to any birthing facilities.
It is useful to consider that the extensibility of all bodily tissues in those childbearing with hEDS/HSD may be greater than in the general childbearing population. During pregnancy, the entire body must be considered since pregnancy hormones such as relaxin influence the body systemically [128]. Those with hEDS/HSD can also present with a lower health related quality of life and greater psychological distress than those in the general population [118], which may be exacerbated by childbearing [10]. Some patients experience a relief in symptoms, particularly dysautonomia and pain. Due to the multifaceted nature of hEDS/HSD, a biopsychosocial approach may be most appropriate in all cases, whereby symptoms such as depression and anxiety are always assessed concurrently with physical symptoms and treated accordingly. In this task, a suite of co-created tools to help perinatal staff support people childbearing with hEDS/HSD may usefully be employed [14].
As connective tissues behave differently for those birthing with hEDS/HSD, it may be useful to investigate outcomes relating to alternate forms of analgesia (e.g., waterbirth) in these populations. Moreover, it is possible that some features of childbearing considered ‘typical’ may actually be associated with hEDS/HSD (e.g., precipitous birth), corresponding with joint hypermobility, skin hyperextensibility and other anomalies of connective tissue [10]. Future research activities could explore these potential links and avoid conflation between the various subtypes of EDS.
Using systematic and co-creation approaches along with a variety of PPI activities, this research is the first of its kind to offer consensus guidelines from an international and multidisciplinary group of experts in the field of childbearing with hEDS/HSD. Limitations include a lack of relevant, larger, longitudinal, and high-quality studies in this field. Those with hEDS/HSD may not have been diagnosed until after their childbearing experience, thus limiting clinical expertise in this area. Further research is required to compare outcomes of interventions designed to address all conditions more prevalent in this childbearing population (e.g., PGP) [128].
Due to conflicting findings and limited research in this area, the guidelines presented have also been guided by patient preference, clinical expertise, practicalities and known biological mechanisms. Inevitably, as evidence-based practitioners, some co-creators have also referenced other published evidence, for example in cases where we have made evidence-based guidelines for the use of complementary and alternative medicines such as acupuncture [69], though the usual contraindications associated with pregnancy still apply [129]. Furthermore, many research findings and guidelines published previously have been made for all EDS types, rather than for individuals with hEDS/HSD specifically. Thus, we have had to review and modify these in light of the wider evidence and expertise within the co-creation team. The quality of some evidence such as clinical case studies was low. Moreover, our scoping review is inherently limited as its focus was to provide breadth rather than depth of information. Many existing guidelines for pregnancy in general populations (e.g., in relation to exercise and pelvic girdle pain) remained relevant and considered equally beneficial with modification(39). Yet in other areas such as cervical insufficiency and preterm labor, further research will be required for more comprehensive guidelines.
A key challenge in conducting this research is that the inheritance of hEDS/HSD has yet to be determined, unlike the other types of EDS. Also, multiple family members may have hEDS/HSD, with significant variability in findings and symptoms among them. Whole exome sequencing has recently identified several genes of interest [65], but at this time there is no genetic testing available. Genetic factors may also play a role where incidence of multiple miscarriages of pregnancy (≥3 with the same partner) in this population is higher(30). Limitations in the literature include the fact that diagnoses of hEDS/HSD are often self-reported, and studies frequently report on all subtypes of EDS as a whole, where some of the rarer types (e.g., vascular) would likely bring increased complications. As hEDS/HSD does represent the vast majority of cases, it is likely representative of the complications reported. Yet even a minority of complications included from one of the rarer types of EDS in an amalgamated cohort study would skews results and thus, the interpretation of risk. Future research could usefully embark upon prospective studies which explore each subtype distinctly. Moreover, despite the risks and issues discussed in the guidelines presented here, it remains important not to over medicalize pregnancy, as many pregnancies in cases of hEDS/HSD are decidedly unremarkable.
Conclusion
The co-creation of evidence-based guidelines in the management of childbearing in cases of hEDS/HSD is justified given the lack of knowledge and awareness demonstrated by healthcare professionals and the profound impacts hEDS/HSD can have upon childbearing populations. Evidence in this area was found to be sparse and somewhat contradictory. Nevertheless, the expert guidelines established here by clinical, academic, and patient experts provide an evidence-informed basis for how care and outcomes may be improved for this childbearing population. In the spirit of evidence-based practice, such guidance could usefully evolve alongside future research in this field.
Other information
SP reports receiving honorariums from the Ehlers-Danlos Society. AH reports receiving honorariums from the Ehlers-Danlos Society. NB reports receiving honorariums from the Ehlers-Danlos Society. Other authors report no conflict of interest. All data extracted has been included in this report.
Supporting information
S1 File. PRISMA-ScR.
PRISMA extension checklist for Scoping Reviews.
https://doi.org/10.1371/journal.pone.0302401.s001
(PDF)
S2 File. Supporting information.
Guidelines for physical therapy.
https://doi.org/10.1371/journal.pone.0302401.s002
(DOCX)
Acknowledgments
The authors wish to thank Dr Maarten (M.F.M.) Engel from the Erasmus MC Medical Library for their support in developing and updating the search strategies. We would also like to thank the experts we consulted throughout, and the Ehlers Danlos Society.
References
- 1.
Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, et al., editors. The 2017 international classification of the Ehlers–Danlos syndromes. American Journal of Medical Genetics Part C: Seminars in Medical Genetics; 2017: Wiley Online Library.
- 2. Blackburn PR, Xu Z, Tumelty KE, Zhao RW, Monis WJ, Harris KG, et al. Bi-allelic alterations in AEBP1 lead to defective collagen assembly and connective tissue structure resulting in a variant of Ehlers-Danlos syndrome. The American Journal of Human Genetics. 2018;102(4):696–705. pmid:29606302
- 3. Tinkle B, Castori M, Berglund B, Cohen H, Grahame R, Kazkaz H, et al. Hypermobile Ehlers–Danlos syndrome (a.k.a. Ehlers–Danlos syndrome Type III and Ehlers–Danlos syndrome hypermobility type): Clinical description and natural history. Am J Med Genet Part C Semin Med Genet. 2017;175(1):48–69. pmid:28145611
- 4. Demmler JC, Atkinson MD, Reinhold EJ, Choy E, Lyons RA, Brophy ST JBo. Diagnosed prevalence of Ehlers-Danlos syndrome and hypermobility spectrum disorder in Wales, UK: a national electronic cohort study and case–control comparison. BMJ Open 2019;9(11):e031365. pmid:31685485
- 5.
Castori M, Tinkle B, Levy H, Grahame R, Malfait F, Hakim A, editors. A framework for the classification of joint hypermobility and related conditions. American Journal of Medical Genetics Part C: Seminars in Medical Genetics; 2017: Wiley Online Library.
- 6. Aubry-Rozier B, Schwitzguebel A, Valerio F, Tanniger J, Paquier C, Berna C, et al. Are patients with hypermobile Ehlers–Danlos syndrome or hypermobility spectrum disorder so different? Rheumatology international. 2021;41(10):1785–94. pmid:34398260
- 7. Pezaro S, Pearce G, Reinhold EJM. Understanding hypermobile Ehlers-Danlos syndrome and Hypermobility Spectrum Disorders in the context of childbearing: An international qualitative study. Midwifery. 2020;88:102749. pmid:32535291
- 8. Hamonet C, Brissot R, Gompel A, Baeza-Velasco C, Guinchat V, Brock I, et al. Ehlers-Danlos syndrome (EDS)-contribution to clinical diagnosis-a prospective study of 853 patients. EC Neurology. 2018;10(6):428–39.
- 9. Hurst BS, Lange SS, Kullstam SM, Usadi RS, Matthews ML, Marshburn PB, et al. Obstetric and gynecologic challenges in women with Ehlers-Danlos syndrome. Obstetrics & Gynecology. 2014;123(3):506–13. pmid:24499752
- 10. Pearce G, Bell L, Pezaro S, Reinhold EJ IJoER, Health P. Childbearing with Hypermobile Ehlers–Danlos Syndrome and Hypermobility Spectrum Disorders: A Large International Survey of Outcomes and Complications. International Journal of Environmental Research and Public Health. 2023;20(20):6957. pmid:37887695
- 11. Pezaro S, Pearce G, Reinhold EJ BJoM. Hypermobile Ehlers-Danlos syndrome during pregnancy, birth and beyond. British Journal of Midwifery. 2018;26(4):217–23.
- 12. Pezaro S, Pearce G, Reinhold EJ BJoM. A clinical update on hypermobile Ehlers-Danlos syndrome during pregnancy, birth and beyond. British Journal of Midwifery. 2021;29(9):492–500.
- 13.
Blagowidow N, editor Obstetrics and gynecology in Ehlers-Danlos syndrome: A brief review and update. American Journal of Medical Genetics Part C: Seminars in Medical Genetics; 2021: Wiley Online Library.
- 14. Pearce G, Bell L, Magee P, Pezaro SJ IJoER, Health P. Co-created solutions for perinatal professionals and childbearing needs for people with hypermobile Ehlers-Danlos syndrome and Hypermobility Spectrum Disorders. International Journal of Environmental Research and Public Health. 2023;20(20):6955. pmid:37887694
- 15. Munn Z, Peters MD, Stern C, Tufanaru C, McArthur A, Aromataris E JBmrm. Systematic review or scoping review? Guidance for authors when choosing between a systematic or scoping review approach. BMC medical research methodology. 2018;18:1–7.
- 16. Nebgen DR, Domchek SM, Kotsopoulos J, de Hullu JA, Crosbie EJ, Paramanandam VS, et al. Care after premenopausal risk-reducing salpingo-oophorectomy in high-risk women: Scoping review and international consensus recommendations. BJOG: An International Journal of Obstetrics & Gynaecology. 2023. pmid:37132126
- 17. Arksey H, O’Malley LJ Ijosrm. Scoping studies: towards a methodological framework. International journal of social research methodology. 2005;8(1):19–32.
- 18. Peters MD, Marnie C, Colquhoun H, Garritty CM, Hempel S, Horsley T, et al. Scoping reviews: reinforcing and advancing the methodology and application. Systematic Reviews. 2021;10(1):1–6.
- 19. Avau B LJ, Van Remoortel H, Vanhove A, Vandekerckhove P, De Buck Development of an evidence-based guideline when evidence is scarce: the importance of a balanced guideline. Cochrane Colloquium digital oral presentation 2019.
- 20.
Pearce G MPObotCWt. Co-creation Solutions and The Three Co’s Framework for applying Co-Creation. Health Education. in press.
- 21. Lewin KJ Josi. Action research and minority problems. 1946;2(4):34–46.
- 22. Baum F, MacDougall C, Smith DJ Joe, health c. Participatory action research. Journal of epidemiology and community health. 2006;60(10):854. pmid:16973531
- 23.
Chevalier JM, Buckles DJ. Participatory action research: Theory and methods for engaged inquiry: Routledge; 2019.
- 24. Arnold J JNDU, Publication. Problem solving—A creative approach. 1956;2.
- 25. Jarrett C, Baxter YC, Boch J, Carrasco C, Cobos Muñoz D, Mauro Dib K, et al. Deconstructing design thinking as a tool for the implementation of a population health initiative. Health Research Policy and Systems. 2022;20(1):91. pmid:35986365
- 26. Hong QN, Pluye P, Fàbregues S, Bartlett G, Boardman F, Cargo M, et al. Mixed methods appraisal tool (MMAT), version 2018. Education for Information. 2018;1148552(10).
- 27. Pluye P, Gagnon M-P, Griffiths F, Johnson-Lafleur JJIjons. A scoring system for appraising mixed methods research, and concomitantly appraising qualitative, quantitative and mixed methods primary studies in mixed studies reviews. International journal of nursing studies. 2009;46(4):529–46.
- 28. Rehfuess EA, Stratil JM, Scheel IB, Portela A, Norris SL, Baltussen R JBGH. The WHO-INTEGRATE evidence to decision framework version 1.0: integrating WHO norms and values and a complexity perspective. 2019;4(Suppl 1):e000844. pmid:30775012
- 29.
Organization WH. Joint United Nations statement on ending discrimination in health care settings. World Health Organization, 2017.
- 30. Armstrong MJ, Mullins CD, Gronseth GS, Gagliardi AR JPo. Recommendations for patient engagement in guideline development panels: a qualitative focus group study of guideline-naïve patients. PloS one. 2017;12(3):e0174329.
- 31. Staniszewska S, Brett J, Simera I, Seers K, Mockford C, Goodlad S, et al. GRIPP2 reporting checklists: tools to improve reporting of patient and public involvement in research. BMJ. 2017;358.
- 32. Kanjwal K, Saeed B, Karabin B, Kanjwal Y, Grubb BPJIp, journal e. Comparative clinical profile of postural orthostatic tachycardia patients with and without joint hypermobility syndrome. Indian pacing and electrophysiology journal. 2010;10(4):173.
- 33. Wright GL, Wen T, Engel DJ, Guglielminotti J, Andrikopoulou M, Booker WA, et al. Delivery Outcomes and Postpartum Readmissions Associated with Ehlers–Danlos Syndrome. American Journal of Perinatology. 2023. pmid:37793432
- 34. Castori M, Morlino S, Dordoni C, Celletti C, Camerota F, Ritelli M, et al. Gynecologic and obstetric implications of the joint hypermobility syndrome (aka Ehlers–Danlos syndrome hypermobility type) in 82 Italian patients. American Journal of Medical Genetics. 2012;158(9):2176–82.
- 35. Hugon-Rodin J, Lebègue G, Becourt S, Hamonet C, Gompel AJOJoRD. Gynecologic symptoms and the influence on reproductive life in 386 women with hypermobility type Ehlers-Danlos syndrome: a cohort study. Orphanet Journal of Rare Diseases. 2016;11(1):1–6.
- 36. Spiegel E, Nicholls-Dempsey L, Czuzoj-Shulman N, Abenhaim HA JTJoM-F, Medicine N. Pregnancy outcomes in women with Ehlers-Danlos Syndrome. The Journal of Maternal-Fetal & Neonatal Medicine. 2022;35(9):1683–9. pmid:32654548
- 37. Knoepp LR, McDermott KC, Muñoz A, Blomquist JL, Handa VL JIuj. Joint hypermobility, obstetrical outcomes, and pelvic floor disorders. International urogynecology journal. 2013;24:735–40. pmid:22898931
- 38. Solak Ö, Turhan-Haktanir N, Köken GJ EJGM. Prevalence of temporomandibular disorders in pregnancy. Eur J Gen Med. 2009;6(4):223–8.
- 39. Sundelin HE, Stephansson O, Johansson K, Ludvigsson JFJAOeGS. Pregnancy outcome in joint hypermobility syndrome and Ehlers–Danlos syndrome. Acta Obstetricia et Gynecologica Scandinavica. 2017;96(1):114–9.
- 40. Sorokin Y, Johnson M, Rogowski N, Richardson D, Evans MJTJoRM. Obstetric and gynecologic dysfunction in the Ehlers-Danlos syndrome. The Journal of Reproductive Medicine. 1994;39(4):281–4.
- 41. Lind J, Wallenburg HS JAOeGS. Pregnancy and the Ehlers-Danlos syndrome: a retrospective study in a Dutch population. Acta Obstetricia et Gynecologica Scandinavica. 2002;81(4):293–300. pmid:11952457
- 42. Karthikeyan A, Venkat-Raman N JOm. Hypermobile Ehlers–Danlos syndrome and pregnancy. Obstetric medicine. 2018;11(3):104–9. pmid:30214474
- 43. Atalla A, Page I JO, Gynecology. Ehlers-Danlos syndrome type III in pregnancy. Obstetrics & Gynecology. 1988;71(3 Pt 2):508–9. pmid:3347446
- 44. Cesare AE, Rafer LC, Myler CS, Brennan KB JTAJoCR. Anesthetic management for Ehlers-Danlos syndrome, hypermobility type complicated by local anesthetic allergy: a case report. The American Journal of Case Reports. 2019;20:39. pmid:30626862
- 45. De Vos M, Nuytinck L, Verellen C, De Paepe A JFd, therapy. Preterm premature rupture of membranes in a patient with the hypermobility type of the Ehlers-Danlos syndrome: A case report. Fetal diagnosis and therapy. 1999;14(4):244–7.
- 46. Fedoruk K, Karen Chong M, Sermer M, Jose C, Carvalho A JCJoA. Anesthetic management of a parturient with hypermobility phenotype but possible vascular genotype Ehlers-Danlos syndrome. Canadian Journal of Anesthesia. 2015;62(12):1308. pmid:26370260
- 47. Garcia-Aguado R, Morales-Rosello J, Rosso MT, Llopis J, Granell M, editors. Ehlers-Danlos syndrome type III and pregnancy: analgesia for delivery. In Annales francaises d’anesthesie et de reanimation 1997 (Vol. 5, No. 16, pp. 523–526).
- 48. Golfier F, Peyrol S, Attia-Sobol J, Marret H, Raudrant D, Plauchu H JCg. Hypermobility type of Ehlers-Danlos syndrome: influence of pregnancies. Clinical genetics. 2001;60(3):240–1. pmid:11595028
- 49. Jones T, Ng C JIJoOA. Anaesthesia for caesarean section in a patient with Ehlers-Danlos syndrome associated with postural orthostatic tachycardia syndrome. International Journal of Obstetric Anesthesia. 2008;17(4):365–9. pmid:18617391
- 50. Kanjwal K, Karabin B, Kanjwal Y, Grubb BP JCR, Practice. Postpartum postural orthostatic tachycardia syndrome in a patient with the joint hypermobility syndrome. Cardiology Research and Practice. 2009;2009.
- 51. Morales-Roselló J, Hernandez-Yago J, Pope M JAog, obstetrics. Type III Ehlers-Danlos syndrome and pregnancy. Archives of gynecology and obstetrics. 1997;261:39–43. pmid:9451523
- 52. Ogawa S, Mariya T, Fujibe Y, Ogawa M, Ikeda K, Mizukami M, et al. Twin pregnancy with untyped Ehlers-Danlos syndrome requiring prompt genetic testing: A case report. Case Reports in Women’s Health. 2022;33:e00384. pmid:35079579
- 53.
Place M, Eden-Green B, editors. Anaesthesia for category 1 caesarean section in a parturient with postural tachycardia syndrome. ANAESTHESIA; 2017: WILEY 111 RIVER ST, HOBOKEN 07030–5774, NJ USA.
- 54. Quak E TR, Pickett J, Bamber J. Head and Neck Cancer in Pregnancy. Obstetric Anaesthetists’ Association; Bournemouth, United Kingdom: International Journal of Obstetric Anesthesia 2013.
- 55. Roop KA, Brost BC JAjoo, gynecology. Abnormal presentation in labor and fetal growth of affected infants with type III Ehlers-Danlos syndrome. American journal of obstetrics and gynecology. 1999;181(3):752–3. pmid:10486495
- 56. Sakala E, Harding MJ TJoRM. Ehlers-Danlos syndrome type III and pregnancy. A case report. The Journal of Reproductive Medicine. 1991;36(8):622–4. pmid:1941807
- 57. Selcer R, Mo L, Yeh J, Osman N, Singh K JMG, Metabolism. Fetal aortic dilation in a mother with clinical diagnosis of Ehlers Danlos syndrome. Molecular Genetics and Metabolism. 2021;132:S311–S2.
- 58. Sizer CF. PHYSIATRIC MANAGEMENT OF PROGRESSIVE FUNCTIONAL DECLINE IN A PREGNANT PATIENT WITH HYPERMOBILITY TYPE EHLERS-DANLOS SYNDROME: A CASE REPORT. American Journal of Physical Medicine & Rehabilitation. 2014:a65–a. 95894889. Language: English. Entry Date: 20140626. Revision Date: 20140626. Publication Type: Article.
- 59. Sood V, Robinson DA, Suri I. Difficult intubation during rapid sequence induction in a parturient with Ehlers-Danlos syndrome, hypermobility type. Int J Obstet Anesth. 2009;18(4):408–12. pmid:19733476
- 60. Taylor DJ, Wilcox I, Russell JK. Ehlers-Danlos syndrome during pregnancy: A case report and review of the literature. OBSTET GYNECOL SURV. 1981;36(6):277–81. pmid:6972498
- 61. Khalil H, Rafi J, Hla TT. A case report of obstetrical management of a pregnancy with hypermobile Ehlers-Danlos syndrome and literature review. Obstet med. 2013;6(2):80–2. pmid:27757162.
- 62. Volkov N, Nisenblat V, Ohel G, Gonen R. Ehlers-Danlos syndrome: Insights on obstetric aspects. Obstet Gynecol Surv. 2007;62(1):51–7. pmid:17176488
- 63. Leduc L, Wasserstrum N JAjop. Successful treatment with the Smith-Hodge pessary of cervical incompetence due to defective connective tissue in Ehlers-Danlos syndrome. American journal of perinatology. 1992;9(01):25–7. pmid:1550628
- 64. Yew KS, Kamps-Schmitt KA, Borge R JAfp. Hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders. American family physician. 2021;103(8):481–92. pmid:33856167
- 65. Alanis-Funes GJ, Lira-Albarrán S, Hernández-Pérez J, Garza-Elizondo MA, Ortíz-López R, Elizondo CV, et al. Genomic Characterization by Whole-Exome Sequencing of Hypermobility Spectrum Disorder. Genes. 2022;13(7):1269. pmid:35886052
- 66.
Levy HP. Hypermobile Ehlers-Danlos Syndrome. 2018.
- 67. Hakim AJ, Cherkas LF, Grahame R, Spector TD, MacGregor AJ JA, Rheumatology R OJotACo. The genetic epidemiology of joint hypermobility: a population study of female twins. Arthritis & Rheumatism: Official Journal of the American College of Rheumatology. 2004;50(8):2640–4. pmid:15334479
- 68.
Chopra P, Tinkle B, Hamonet C, Brock I, Gompel A, Bulbena A, et al., editors. Pain management in the Ehlers–Danlos syndromes. American Journal of Medical Genetics Part C: Seminars in Medical Genetics; 2017: Wiley Online Library.
- 69. Chang S JTJoO, Gynecology. The meridian system and mechanism of acupuncture—a comparative review. Part 2: mechanism of acupuncture analgesia. Taiwanese Journal of Obstetrics and Gynecology. 2013;52(1):14–24. pmid:23548213
- 70. Morissette R, Chen W, Perritt AF, Dreiling JL, Arai AE, Sachdev V, et al. Broadening the spectrum of Ehlers Danlos syndrome in patients with congenital adrenal hyperplasia. The Journal of Clinical Endocrinology & Metabolism. 2015;100(8):E1143–E52. pmid:26075496
- 71. Marino R, Moresco A, Perez Garrido N, Ramirez P, Belgorosky A JFiE. Congenital adrenal hyperplasia and ehlers-danlos syndrome. Frontiers in Endocrinology. 2022;13:803226. pmid:35282436
- 72. Casanova EL, Sharp JL, Edelson SM, Kelly DP, Sokhadze EM, Casanova MF Jb. Immune, autonomic, and endocrine dysregulation in autism and Ehlers-Danlos syndrome/hypermobility spectrum disorders versus unaffected controls. bioRxiv. 2019:670661.
- 73. Morgan K, Smith A, Blitshteyn SJ Ijowsh. POTS and Pregnancy: A Review of Literature and Recommendations for Evaluation and Treatment. International Journal of Women’s Health 2022:1831–47. pmid:36590760
- 74. Morgan K, Chojenta C, Tavener M, Smith A, Loxton D JAN. Postural orthostatic tachycardia syndrome during pregnancy: a systematic review of the literature. Autonomic Neuroscience 2018;215:106–18. pmid:29784553
- 75. Dorff SR, Afrin LB JJoO, Gynaecology. Mast cell activation syndrome in pregnancy, delivery, postpartum and lactation: a narrative review. Journal of Obstetrics and Gynaecology. 2020;40(7):889–901.
- 76. Woidacki K, Zenclussen AC, Siebenhaar F JFii. Mast cell-mediated and associated disorders in pregnancy: a risky game with an uncertain outcome? Frontiers in immunology. 2014;5:231. pmid:24904581
- 77.
Brock I, Prendergast W, Maitland A, editors. Mast cell activation disease and immunoglobulin deficiency in patients with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorder. American Journal of Medical Genetics Part C: Seminars in Medical Genetics; 2021: Wiley Online Library.
- 78.
Glayzer JE, McFarlin BL, Castori M, Suarez ML, Meinel MC, Kobak WH, et al., editors. High rate of dyspareunia and probable vulvodynia in Ehlers–Danlos syndromes and hypermobility spectrum disorders: An online survey. American Journal of Medical Genetics Part C: Seminars in Medical Genetics; 2021: Wiley Online Library.
- 79. Artoni A, Bassotti A, Abbattista M, Marinelli B, Lecchi A, Gianniello F, et al. Hemostatic abnormalities in patients with Ehlers–Danlos syndrome. Journal of Thrombosis and Haemostasis. 2018;16(12):2425–31. pmid:30312027
- 80. Ainsworth SR, Aulicino PL JCO, Research® R. A survey of patients with Ehlers-Danlos syndrome. Clinical Orthopaedics and Related Research®. 1993;286:250–6. pmid:8425354
- 81. Mardy AH, Chetty SP, Norton ME JPD. Maternal genetic disorders and fetal development. Prenatal Diagnosis. 2020;40(9):1056–65. pmid:32010984
- 82. Underhill L, Barbarita C, Collis S, Tucker R, Lechner B JRS. Association of maternal versus fetal ehlers-danlos syndrome Status with poor pregnancy outcomes. Reproductive Sciences. 2022;29(12):3459–64. pmid:35676499
- 83. Jesudas R, Chaudhury A, Laukaitis CM JH. An update on the new classification of Ehlers-Danlos syndrome and review of the causes of bleeding in this population. Haemophilia. 2019;25(4):558–66. pmid:31329366
- 84. Ahlqvist K, Bjelland EK, Pingel R, Schlager A, Nilsson-Wikmar L, Kristiansson P JBMD. The Association of Self-Reported Generalized Joint Hypermobility with pelvic girdle pain during pregnancy: a retrospective cohort study. BMC musculoskeletal disorders. 2020;21:1–10. pmid:32689990
- 85. Gazit Y, Jacob G, Grahame R. Ehlers-Danlos Syndrome-Hypermobility Type: A Much Neglected Multisystemic Disorder. Rambam Maimonides Medical Journal. 2016. pmid:27824552.
- 86. Enix DE, Mayer JM JP. Sacroiliac joint hypermobility biomechanics and what it means for health care providers and patients. PM&R. 2019;11:S32–S9. pmid:31025539
- 87. Vleeming A, Albert H, Ostgaard H, Stuge B, Sturesson B JAoawbeo, accessed April. European guidelines on the diagnosis and treatment of pelvic girdle pain [European Commission Research Directorate General Web site]. 2005. 2008.
- 88. Simmonds J, Castori M, Pacey V, Keer R. Advancing practice in joint hypermobility syndrome/ ehlers danlos syndrome-hypermobility type. Manual Therapy. 2016;25:e21–e2.
- 89. Simmonds JV, Keer RJ JMt. Hypermobility and the hypermobility syndrome, part 2: assessment and management of hypermobility syndrome: illustrated via case studies. Manual Therapy 2008;13(2):e1–e11. pmid:18221908
- 90. Ferrell WR, Tennant N, Sturrock RD, Ashton L, Creed G, Brydson G, et al. Amelioration of symptoms by enhancement of proprioception in patients with joint hypermobility syndrome. Official Journal of the American College of Rheumatology. 2004;50(10):3323–8. pmid:15476239
- 91. Daman M, Shiravani F, Hemmati L, Taghizadeh SJ JoB, Therapies M. The effect of combined exercise therapy on knee proprioception, pain intensity and quality of life in patients with hypermobility syndrome: a randomized clinical trial. Journal of Bodywork and Movement Therapies. 2019;23(1):202–5. pmid:30691753
- 92. Sahin N, Baskent A, Cakmak A, Salli A, Ugurlu H, Berker E JRi. Evaluation of knee proprioception and effects of proprioception exercise in patients with benign joint hypermobility syndrome. Rheumatology international. 2008;28:995–1000. pmid:18368409
- 93. Toprak Celenay S, Ozer Kaya D JRi. Effects of spinal stabilization exercises in women with benign joint hypermobility syndrome: a randomized controlled trial. Rheumatology international. 2017;37:1461–8. pmid:28361275
- 94. Reychler G, De Backer MM, Piraux E, Poncin W, Caty G JAJoMGPA. Physical therapy treatment of hypermobile Ehlers–Danlos syndrome: a systematic review. American Journal of Medical Genetics Part A. 2021;185(10):2986–94. pmid:34145717
- 95. Goom T, Donnelly G, Brockwell E JAP. Returning to running postnatal–guidelines for medical, health and fitness professionals managing this population. Sports Medicine. 2019.
- 96. Quartarone G JMg. Gastroesophageal reflux in pregnancy: a systematic review on the benefit of raft forming agents. Minerva ginecologica. 2013;65(5):541–9. pmid:24096290
- 97. Gerson LB JG, Hepatology. Treatment of gastroesophageal reflux disease during pregnancy. Gastroenterology & Hepatology. 2012;8(11):763. pmid:24672414
- 98. Bourne KM, Nerenberg KA, Stiles LE, Shibao CA, Okamoto LE, Garland EM, et al. Symptoms of postural orthostatic tachycardia syndrome in pregnancy: a cross-sectional, community-based survey. An International Journal of Obstetrics & Gynaecology. 2023. pmid:36908200
- 99. Shakur H, Roberts I, Fawole B, Chaudhri R, El-Sheikh M, Akintan A, et al. Effect of early tranexamic acid administration on mortality, hysterectomy, and other morbidities in women with post-partum haemorrhage (WOMAN): an international, randomised, double-blind, placebo-controlled trial. The Lancet. 2017;389(10084):2105–16. pmid:28456509
- 100. Wiesmann T, Castori M, Malfait F, Wulf H. Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s). Orphanet Journal of Rare Diseases. 2014;9(1):109-. 103852351. Language: English. Entry Date: 20150410. Revision Date: 20211030. Publication Type: journal article. pmid:25053156
- 101. Santos FF, Lourenço BM, Souza MB, Maia LB, Oliveira VC, Oliveira MX JP. Prevention of low back and pelvic girdle pain during pregnancy: a systematic review and meta-analysis of randomised controlled trials with GRADE recommendations. Physiotherapy. 2023;118:1–11. pmid:36288631
- 102. Aldabe D, Lawrenson P, Sullivan J, Hyland G, Bussey MD, Hammer N, et al. Management of women with pregnancy-related pelvic girdle pain: An international Delphi study. Physiotherapy 2022;115:66–84. pmid:35202976
- 103. Pulsifer J, Britnell S, Sim A, Adaszynski J, Dufour S JBJoSM. Reframing beliefs and instiling facts for contemporary management of pregnancy-related pelvic girdle pain. BMJ Publishing Group Ltd and British Association of Sport and Exercise Medicine; 2022. p. 1262–5. pmid:35948414
- 104. Arendt-Nielsen L, Kaalund S, Bjerring P, Høgsaa B JAAS. Insufficient effect of local analgesics in Ehlers Danlos type III patients (connective tissue disorder). Acta Anaesthesiologica Scandinavica. 1990;34(5):358–61. pmid:2389651
- 105. Dolan P, Sisko F, Riley E JTJotASoA. Anesthetic considerations for Ehlers-Danlos syndrome. The Journal of the American Society of Anesthesiologists. 1980;52(3):266–9. pmid:7369516
- 106. Schievink WI, Gordon OK, Tourje JJN. Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study. Neurosurgery. 2004;54(1):65–71. pmid:14683542
- 107. Schoenfeld E, Hendler S, Carroll I, Nguyen LA JOjotACoG, ACG. Correlation between ehlers-danlos syndrome (EDS), spontaneous and cerebral spinal fluid (CSF) leaks, and GI motility disorders: 2559. Official journal of the American College of Gastroenterology| ACG. 2017;112:S1397.
- 108. Hakim A, Grahame R. Joint hypermobility. Best Pract Res Clin Rheumatol. 2003;17(6):989–1004. pmid:15123047
- 109. Castori M, Camerota F, Celletti C, Danese C, Santilli V, Saraceni VM, et al. Natural history and manifestations of the hypermobility type Ehlers-Danlos syndrome: a pilot study on 21 patients. Am J Med Genet A. 2010;152A(3):556–64. pmid:20140961.
- 110. Hastings J, Forster JE, Witzeman K. Joint Hypermobility among Female Patients Presenting with Chronic Myofascial Pelvic Pain. PM R. 2019;11(11):1193–9. pmid:30729750
- 111. Mogren IM, Pohjanen AI JS. Low back pain and pelvic pain during pregnancy: prevalence and risk factors. Spine. 2005;30(8):983–91. pmid:15834344
- 112. McIntosh LJ, Stanitski DF, Mallett VT, Frahm J, Richardson DA, Evans MI. Ehlers-Danlos syndrome: Relationship between joint hypermobility, urinary incontinence, and pelvic floor prolapse. GYNECOL OBSTET INVEST. 1996;41(2):135–9. pmid:8838976
- 113. Lammers K, Lince SL, Spath MA, van Kempen LC, Hendriks JC, Vierhout ME, et al. Pelvic organ prolapse and collagen-associated disorders. International urogynecology journal. 2012;23:313–9. pmid:21811768
- 114. Conti R, Zanchi C, Barbi E. A floppy infant without lingual frenulum and kyphoscoliosis: Ehlers Danlos syndrome case report. Italian Journal of Pediatrics. 2021;47(1):1–4. 148676295. Language: English. Entry Date: 20210217. Revision Date: 20210217. Publication Type: Article. pmid:33579342
- 115. Holick M, Hossein-Nezhad A, Tabatabaei F JD-e. Multiple fractures in infants who have Ehlers-Danlos/hypermobility syndrome and or vitamin D deficiency: A case series of 72 infants whose parents were accused of child abuse and neglect. Dermato-endocrinology. 2017;9(1):e1279768. pmid:29511428
- 116. Tofts LJ, Simmonds J, Schwartz SB, Richheimer RM, O’Connor C, Elias E, et al. Pediatric joint hypermobility: a diagnostic framework and narrative review. Orphanet journal of rare diseases. 2023;18(1):104. pmid:37143135
- 117. Francis J, Dickton DD. Considerations for lactation with Ehlers-Danlos syndrome: a narrative review. Int Breastfeed J. 2022;17(1):4. pmid:34983567
- 118. Orenius T, Kautiainen H, Louhi M, Montin L, Bulbena A, Lindgren K-A JSO. Health-Related Quality of Life and Psychological Distress in Patients With Hypermobility Type Ehlers-Danlos Syndrome. Sage Open. 2022;12(2):21582440221091237.
- 119. Halverson CM, Penwell HL, Francomano CA JS-QRiH. Clinician-associated traumatization from difficult medical encounters: Results from a qualitative interview study on the Ehlers-Danlos Syndromes. SSM-Qualitative Research in Health. 2023;3:100237. pmid:37426705
- 120. Kindgren E, Quiñones Perez A, Knez R JNd, treatment. Prevalence of ADHD and autism spectrum disorder in children with hypermobility spectrum disorders or hypermobile Ehlers-Danlos syndrome: a retrospective study. Neuropsychiatric disease and treatment. 2021:379–88. pmid:33603376
- 121. Csecs JL, Iodice V, Rae CL, Brooke A, Simmons R, Quadt L, et al. Joint hypermobility links neurodivergence to dysautonomia and pain. Frontiers in psychiatry. 2022;12:786916. pmid:35185636
- 122.
Bulbena-Cabré A, Baeza-Velasco C, Rosado-Figuerola S, Bulbena A, editors. Updates on the psychological and psychiatric aspects of the Ehlers–Danlos syndromes and hypermobility spectrum disorders. American Journal of Medical Genetics Part C: Seminars in Medical Genetics; 2021: Wiley Online Library.
- 123. Eftekhari H, Maddock H, Pearce G, Raza S, Kavi L, Lim P, et al. Understanding the future research needs in Postural Orthostatic Tachycardia Syndrome (POTS): Evidence mapping the POTS adult literature. Autonomic Neuroscience. 2021;233:102808. pmid:33901811
- 124. Dutta I, Wilson H, Oteri O. Pregnancy and delivery in ehlers-danlos syndrome (hypermobility type): review of the literature. Obstet Gynecol Int. 2011;2011:306413. pmid:21765833.
- 125. Kang J, Hanif M, Mirza E, Jaleel S. Ehlers-Danlos Syndrome in Pregnancy: A Review. Eur J Obstet Gynecol Reprod Biol. 2020;255:118–23. pmid:33113401
- 126. Alrifai N, Alhuneafat L, Jabri A, Khalid MU, Tieliwaerdi X, Sukhon F, et al. Pregnancy and fetal outcomes in patients with Ehlers-Danlos syndrome: a nationally representative analysis. Current Problems in Cardiology. 2023;48(7):101634. pmid:36806636
- 127. Brock I, Chopra P, Maitland A, Francomano C JMG, Metabolism. Frequency and co-occurrence of comorbidities in the Ehlers-Danlos syndromes. Molecular Genetics and Metabolism. 2021;132:S194.
- 128. Ali A, Andrzejowski P, Kanakaris NK, Giannoudis PV. Pelvic girdle pain, hypermobility spectrum disorder and hypermobility-type ehlers-danlos syndrome: A narrative literature review. J Clin Med. 2020;9(12):1–26. pmid:33317183
- 129. Deadman P, Al-Khafaji M, Baker KJ. A manual of acupuncture. 2007.