The most common comorbidities in patients with Ehlers-Danlos syndrome: a 15-year nationwide population-based cohort study

Leganger, Julie; Fonnes, Siv; Søborg, Marie Louise Kulas; Rosenberg, Jacob; Burcharth, Jakob

doi:10.1080/09638288.2020.1761890

Cited by 15 publications

(12 citation statements)

References 25 publications

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“…In a Danish population registry study (n = 48,019, median age 31 IQR 16–43) patients presenting at hospital with all types of EDS (ICD10 Q79.6, n = 1319) and not including GJH/HSD had musculoskeletal contusions and sprains at more than 10% frequency. Meniscal injuries and “unspecified” knee injuries were commoner in people with EDS than controls [ 26 ].…”

Section: Discussion/observationsmentioning

confidence: 99%

“…In a general population cohort [ 19 ] participants at 17.8 years who had a Beighton score ≥ 6–9 at 13.8 years demonstrated a weak suggestion of a higher risk of chronic regional pain and chronic widespread pain (OR 1.84 (CI 0.97–3.50, p = 0.062) and 2.27 (CI 1.22–4.22, p = 0.01) respectively). Significantly increased rates of extremity pain and abdominal pain were reported in patients across the lifespan with EDS (ICD-10 DQ796) (n = 1319) compared to database controls (n = 46,700); about 400 of the EDS patients were diagnosed in childhood [ 26 ]. Pain-related disability was found in the Sydney cohort [ 17 ] as well as 47 10–20-year-olds with hypermobility (Beighton score 4–9).…”

Section: Discussion/observationsmentioning

confidence: 99%

“…No studies have reported the prevalence of FGID in hypermobile children in comparison to controls. However, significantly increased rates of FGID were reported in a lifespan registry cohort, in 13% of the EDS population compared to 3.9% of controls ( p = < 0.001) [ 26 ]. The group consensus was these conditions were important comorbidities.…”

Section: Discussion/observationsmentioning

confidence: 99%

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Pediatric joint hypermobility: a diagnostic framework and narrative review

Tofts

Simmonds

Schwartz

et al. 2023

Orphanet J Rare Dis

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Background Hypermobile Ehlers–Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD) are debilitating conditions. Diagnosis is currently clinical in the absence of biomarkers, and criteria developed for adults are difficult to use in children and biologically immature adolescents. Generalized joint hypermobility (GJH) is a prerequisite for hEDS and generalized HSD. Current literature identifies a large proportion of children as hypermobile using a Beighton score ≥ 4 or 5/9, the cut off for GJH in adults. Other phenotypic features from the 2017 hEDS criteria can arise over time. Finally, many comorbidities described in hEDS/HSD are also seen in the general pediatric and adolescent population. Therefore, pediatric specific criteria are needed. The Paediatric Working Group of the International Consortium on EDS and HSD has developed a pediatric diagnostic framework presented here. The work was informed by a review of the published evidence. Observations The framework has 4 components, GJH, skin and tissue abnormalities, musculoskeletal complications, and core comorbidities. A Beighton score of ≥ 6/9 best identifies children with GJH at 2 standard deviations above average, based on published general population data. Skin and soft tissue changes include soft skin, stretchy skin, atrophic scars, stretch marks, piezogenic papules, and recurrent hernias. Two symptomatic groups were agreed: musculoskeletal and systemic. Emerging comorbid relationships are discussed. The framework generates 8 subgroups, 4 pediatric GJH, and 4 pediatric generalized hypermobility spectrum disorders. hEDS is reserved for biologically mature adolescents who meet the 2017 criteria, which also covers even rarer types of Ehlers–Danlos syndrome at any age. Conclusions This framework allows hypermobile children to be categorized into a group describing their phenotypic and symptomatic presentation. It clarifies the recommendation that comorbidities should be defined using their current internationally accepted frameworks. This provides a foundation for improving clinical care and research quality in this population.

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Section: Discussion/observationsmentioning

confidence: 99%

Section: Discussion/observationsmentioning

confidence: 99%

Section: Discussion/observationsmentioning

confidence: 99%

See 1 more Smart Citation

Pediatric joint hypermobility: a diagnostic framework and narrative review

Tofts

Simmonds

Schwartz

et al. 2023

Orphanet J Rare Dis

View full text Add to dashboard Cite

show abstract

“…These include Postural Tachycardia Syndrome (PoTS), 4 Mast Cell Activation Syndrome (MCAS), 5 gastrointestinal issues, and bladder and bowel problems. 6 These comorbidities can play an important role when it comes to rehabilitation planning. 7 Comorbidities are associated with worse health outcomes, more complex clinical management, and increased healthcare costs.…”

Section: Introductionmentioning

confidence: 99%

“…Additional consideration must also be given to the comorbidities that exist alongside the main condition due to its multisystemic nature. These include Postural Tachycardia Syndrome (PoTS), 4 Mast Cell Activation Syndrome (MCAS), 5 gastrointestinal issues, and bladder and bowel problems 6 . These comorbidities can play an important role when it comes to rehabilitation planning 7 .…”

Section: Introductionmentioning

confidence: 99%