Ehlers–Danlos syndrome hypermobility type: a possible unifying concept for various functional somatic syndromes

Castori, Marco; Celletti, Claudia; Camerota, Filippo

doi:10.1007/s00296-011-2275-2

Cited by 18 publications

(21 citation statements)

References 14 publications

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“…Another hypothesis proposes that an underlying dysautonomia leads to visceral and somatic hypersensitivity which then results in GI and somatic symptoms. 40 In our study autonomic factors did appear to be involved in the association between EDS-HT and GI symptoms, however, the mechanism for this remains unknown, as does the underlying etiology for the autonomic symptoms. Future physiological studies investigating autonomic and GI function, and assessing for visceral and somatic hypersensitivity will be paramount in testing these hypotheses and in bringing us a step closer to understanding the etiology of EDS-HT.…”

Section: Discussioncontrasting

confidence: 50%

“…This would suggest that GI symptoms in our students who were not seeking medical attention were not a confounding effect of opiates or pain phenotypes. Another hypothesis proposes that an underlying dysautonomia leads to visceral and somatic hypersensitivity which then results in GI and somatic symptoms . In our study autonomic factors did appear to be involved in the association between EDS‐HT and GI symptoms, however, the mechanism for this remains unknown, as does the underlying etiology for the autonomic symptoms.…”

Section: Discussioncontrasting

confidence: 49%

See 1 more Smart Citation

The association between Ehlers‐Danlos syndrome—hypermobility type and gastrointestinal symptoms in university students: a cross‐sectional study

Fikree

Aktar

Morris

et al. 2016

Neurogastroenterology Motil

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Section: Discussioncontrasting

confidence: 50%

Section: Discussioncontrasting

confidence: 49%

The association between Ehlers‐Danlos syndrome—hypermobility type and gastrointestinal symptoms in university students: a cross‐sectional study

Fikree

Aktar

Morris

et al. 2016

Neurogastroenterology Motil

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“…Among the multifaceted clinical implications of JHS/EDS-HT, there are some gynecologic and obstetric aspects [Dickson et al, 2011;Molloholli, 2011], which are noteworthy due to the unexpected predominance of female patients [Castori et al, 2011b]. Actual knowledge is essentially limited to a handful of case reports [Atalla and Page, 1988;Thornton et al, 1988;Rochelson et al, 1991;Sakala and Harding, 1991;Morales-Rosell o et al, 1997;De Vos et al, 1999;Jones and Ng, 2008;Dutta et al, 2011] and case series mixing various forms of EDS, mainly EDS-HT, EDS classic, and vascular types [Sorokin et al, 1994;McIntosh et al, 1995;Lind and Wallenburg, 2002].…”

Section: Introductionmentioning

confidence: 99%

Gynecologic and obstetric implications of the joint hypermobility syndrome (a.k.a. Ehlers–Danlos syndrome hypermobility type) in 82 Italian patients

Castori

Morlino

Dordoni

et al. 2012

American J of Med Genetics Pt A

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Joint hypermobility syndrome (JHS) emerges as likely the most common clinical form of Ehlers-Danlos syndrome. Given the striking predominance of affected women, practitioners often face gynecologic and obstetric issues. However, their decisions are still based on personal experience rather than literature due to the lack of a consistent body of evidence. We collected a set of gynecologic and obstetric features in 82 post-puberal women with JHS attending two Italian centers. Common gynecologic findings were dysmenorrhea (82.9%), meno/metrorrhagias (53.7%), irregular menses (46.3%), and dispareunia/vulvodinia (31.7%). Forty women were nulliparous and 42 had one or more pregnancy for a total of 93 diagnosed conceptions. Of them, 16.1% were spontaneous abortions, 6.5% voluntary interruptions, 10.7% preterm deliveries, and 66.7% deliveries at term. Overall outcome of proceeding pregnancies was good with no stillbirth and fetal/neonatal hypoxic/ischemic event. Non-operative vaginal delivery was registered in 72.2%, forceps/vacuum use in 5.5% and cesarean in 22.3%. Local/total anesthesia was successfully performed in 17 pregnancies without any problem. Major post-partum complications included abnormal scar formation after cesarean or episiotomy (46.1%), hemorrhage (19.4%), pelvic prolapses (15.3%), deep venous thrombosis (4.2%), and coccyx dislocation (1.4%). Prolapses were the most clinically relevant complication and associated with episiotomy. Gathered data were discussed for practically oriented considerations.

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“…Among the six major forms [1], the classic and the hypermobility (EDS-HT) types are considered the most common. EDS-HT typically features joint laxity and related complications, chronic/recurrent limb pain, and minor skin involvement [1], although its extended clinical spectrum covers a wide variety of functional somatic syndromes [2]. An international panel of experts now considers EDS-HT one and the same as joint hypermobility syndrome (JHS) [3], and this overlap is particularly evident in adulthood.…”

Section: Introductionmentioning

confidence: 99%

Evaluation of Kinesiophobia and Its Correlations with Pain and Fatigue in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type

Celletti

Castori

Torre

et al. 2013

BioMed Research International

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Ehlers-Danlos syndrome hypermobility type a. k. a. joint hypermobility syndrome (JHS/EDS-HT) is a hereditary musculoskeletal disorder associating generalized joint hypermobility with chronic pain. Anecdotal reports suggest a prominent role for kinesiophobia in disease manifestations, but no study has systematically addressed this point. Objective. To investigate the impact of kinesiophobia and its relationship with pain, fatigue, and quality of life in JHS/EDS-HT. Design. Cross-sectional study. Subjects/Patients. 42 patients (40 female and 2 male) with JHS/EDS-HT diagnosis following standardized diagnostic criteria were selected. Methods. Disease features were analyzed by means of specific questionnaires and scales evaluating kinesiophobia, pain, fatigue, and quality of life. The relationships among variables were investigated using the Spearman bivariate analysis. Results. Kinesiophobia resulted predominantly in the patients' sample. The values of kinesiophobia did not correlate with intensity of pain, quality of life, and (or) the single component of fatigue. A strong correlation was discovered between kinesiophobia and general severity of fatigue. Conclusions. In JHS/EDS-HT, the onset of pain-avoiding strategies is related to the presence of pain but not to its intensity. The clear-cut correlation between kinesiophobia and severity of fatigue suggests a direct link between musculoskeletal pain and fatigue. In JHS/EDS-HT, the underlying mechanism is likely to be facilitated by primary disease characteristics, including hypotonia.

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Ehlers–Danlos syndrome hypermobility type: a possible unifying concept for various functional somatic syndromes

Cited by 18 publications

References 14 publications

The association between Ehlers‐Danlos syndrome—hypermobility type and gastrointestinal symptoms in university students: a cross‐sectional study

The association between Ehlers‐Danlos syndrome—hypermobility type and gastrointestinal symptoms in university students: a cross‐sectional study

Gynecologic and obstetric implications of the joint hypermobility syndrome (a.k.a. Ehlers–Danlos syndrome hypermobility type) in 82 Italian patients

Evaluation of Kinesiophobia and Its Correlations with Pain and Fatigue in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type

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