Systemic manifestations of Ehlers-Danlos syndrome

Song, Bo; Yeh, Peter C.; Harrell, John S.

doi:10.1080/08998280.2020.1805714

Cited by 18 publications

(18 citation statements)

References 27 publications

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Section: Mcad-related Disorders and Heds/hsdmentioning

confidence: 99%

“…Headache has been shown to be prevalent in hEDS/HSD patients when compared to controls [ 20 , 31 – 34 ]. In a study by Song et al, 67% of hEDS patients reported symptoms of headache [ 20 ]. While the relationship between headaches and hEDS/HSD patients found in this study may be due to multifactorial etiologies associated with hEDS/HSD such as craniocervical junction instability, temporomandibular joint instability, and dysautonomia, MC-related diseases should be a consideration [ 20 , 31 – 34 ].…”

Section: Mcad-related Disorders and Heds/hsdmentioning

confidence: 99%

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Association of mast-cell-related conditions with hypermobile syndromes: a review of the literature

et al. 2022

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Ehlers–Danlos syndrome (EDS) is a group of related connective tissue disorders consisting of 13 subtypes, each with its own unique phenotypic and genetic variation. The overlap of symptoms and multitude of EDS variations makes it difficult for patients to achieve a diagnosis early in the course of their disease. The most common form, hypermobile type EDS (hEDS) and its variant, hypermobile spectrum disorder (HSD), are correlated with rheumatologic and inflammatory conditions. Evidence is still needed to determine the pathophysiology of hEDS; however, the association among these conditions and their prevalence in hEDS/HSD may be explained through consideration of persistent chronic inflammation contributing to a disruption of the connective tissue. Aberrant mast cell activation has been shown to play a role in disruption of connective tissue integrity through activity of its mediators including histamine and tryptase which affects multiple organ systems resulting in mast cell activation disorders (MCAD). The overlap of findings associated with MCAD and the immune-mediated and rheumatologic conditions in patients with hEDS/HSD may provide an explanation for the relationship among these conditions and the presence of chronic inflammatory processes in these patients. It is clear that a multidisciplinary approach is required for the treatment of patients with EDS. However, it is also important for clinicians to consider the summarized symptoms and MCAD-associated characteristics in patients with multiple complaints as possible manifestations of connective tissue disorders, in order to potentially aid in establishing an early diagnosis of EDS. Supplementary Information The online version contains supplementary material available at 10.1007/s12026-022-09280-1.

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Section: Mcad-related Disorders and Heds/hsdmentioning

confidence: 99%

Section: Mcad-related Disorders and Heds/hsdmentioning

confidence: 99%

Association of mast-cell-related conditions with hypermobile syndromes: a review of the literature

et al. 2022

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“…Conditions with increased fascia mobility include Ehlers-Danlos Syndromes (EDS) and Hypermobility Spectrum Disorders (HSD), a group of conditions involving connective tissue that can be inherited and are generally characterized by joint hypermobility, with or without skin hyperextensibility, and tissue fragility. Patients with EDS have high rates of musculoskeletal pain affecting the back and large joints (shoulder, knee, and hip), suggesting that the larger, higher activity-bearing joints with greater ranges of motion may be more susceptible to injury and pain, with a lower prevalence of pain in smaller, less mobile joints [ 51 , 52 ]. Although most of the known musculoskeletal manifestations of these syndromes relate to joints, the involvement of other connective tissues, including fasciae, may be relevant to the frequent occurrence of chronic musculoskeletal pain in these patients.…”

Section: Does Generalized Hypo- or Hypermobility Predispose One To Or Protect From Myofascial Pain?mentioning

confidence: 99%

Fascia Mobility, Proprioception, and Myofascial Pain

Langevin

2021

Life

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The network of fasciae is an important part of the musculoskeletal system that is often overlooked. Fascia mobility, especially along shear planes separating muscles, is critical for musculoskeletal function and may play an important, but little studied, role in proprioception. Fasciae, especially the deep epimysium and aponeuroses, have recently been recognized as highly innervated with small diameter fibers that can transmit nociceptive signals, especially in the presence of inflammation. Patients with connective tissue hyper- and hypo-mobility disorders suffer in large number from musculoskeletal pain, and many have abnormal proprioception. The relationships among fascia mobility, proprioception, and myofascial pain are largely unstudied, but a better understanding of these areas could result in improved care for many patients with musculoskeletal pain.

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“…Foundationally, EDS causes dysfunction in collagen fibers and while subtypes describe specific body systems or mechanisms that are more directly affected [ 7 ], all patients with EDS, to some extent, experience generalized, broad systemic complaints. Tissue laxity (often identified as “stretchy skin”), joint hypermobility (frequently identified as “loose / unstable joints”), difficulties with proprioception, or acute awareness of where body parts are in space, and other generalized systemic problems such as intestinal dysmotility, impaired healing, and inappropriate scarring can be present [ 8 ]. Due to the proprioception abnormalities, patients with EDS are frequently unaware their joints are sliding out of alignment or into a dangerously hyper-flexed or hyper-extended state until they experience pain.…”

Section: Introductionmentioning

confidence: 99%

“…While it was once thought to be an extraordinarily rare condition, the prevalence of EDS using newer estimation models could be as common as 194 per 100,000 in 2016 / 2017 [ 11 ]. As genetic research has increased greatly in this area, the understanding of EDS subtypes and variations has become an expanding field of study [ 8 ]. There can be an overlap of signs and symptoms with other connective tissue disorders and, challengingly, some current definitions for clinical criteria are non-specific.…”

Section: Introductionmentioning

confidence: 99%

Considerations for lactation with Ehlers-Danlos syndrome: a narrative review

Francis

Dickton²

2022

Int Breastfeed J

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Background Ehlers-Danlos syndrome (EDS) is a rare genetic connective tissue condition that is poorly understood in relation to lactation. As diagnostic methods improve, prevalence has increased. EDS, a disorder that impacts connective tissue, is characterized by skin extensibility, joint hypermobility, and fragile tissue which can affect every organ and body system leading to complications during pregnancy, delivery, and the postpartum period. Traits of this disease can cause mild to severe physiologic and functional obstacles during lactation. Unfortunately, there is little clinical evidence and minimal guidance for lactation management, and providers may feel uncomfortable and hesitant to address these concerns with patients due to a lack of readily available resources on the subject and inexperience with such patients. This narrative review describes and discusses the types of EDS, identifying symptoms, considerations, and precautions for care providers to implement during lactation and breastfeeding. Methods An electronic search of relevant citations was conducted using the databases Cochrane, PubMed, and Google Scholar from 1 January 2000 to 1 November 2021. Search terms used were Ehlers-Danlos syndrome, Hypermobility Syndrome, breastfeeding, lactation, breastmilk expression, breastmilk collection, human milk expression, human milk collection, and infant feeding. The search of these databases yielded zero results. As no research articles on EDS were directly related to lactation, this narrative review includes articles found that related to the health of mothers relevant to maternal function during lactation. Discussion For the healthcare provider, identifying characteristics of EDS can improve the management of lactation challenges. Mothers may experience generalized symptoms from gastrointestinal distress to fatigue or chronic pain, while they also may suffer from more specific joint complaints and injuries, such as dislocations / subluxations, or skin fragility. Such obstacles can generate impediments to breastfeeding and create unique challenges for breastfeeding mothers with EDS. Unfortunately, new mothers with these symptoms may have them overlooked or not addressed, impacting a mother’s ability to meet her breastfeeding intentions. While there are some published research manuscripts on EDS and pregnancy, there is a lack of information regarding breastfeeding and lactation. Additional research is needed to help guide EDS mothers to achieve their breastfeeding intentions.

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Systemic manifestations of Ehlers-Danlos syndrome

Cited by 18 publications

References 27 publications

Association of mast-cell-related conditions with hypermobile syndromes: a review of the literature

Association of mast-cell-related conditions with hypermobile syndromes: a review of the literature

Fascia Mobility, Proprioception, and Myofascial Pain

Considerations for lactation with Ehlers-Danlos syndrome: a narrative review

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