Prevalence of joint hypermobility syndromes in pediatric postural orthostatic tachycardia syndrome
Abstract
Introduction Patients with postural orthostatic tachycardia syndrome (POTS) have been shown to exhibit comorbid joint hypermobility manifested as Ehlers-Danlos syndrome (EDS) or hypermobility spectrum disorder (HSD). The prevalence of EDS and HSD in POTS has been demonstrated in smaller studies combining adult and pediatric patients. We examined a large series of pediatric patients to determine their prevalence in children with POTS. Materials and methods Patients 18 years old, or less, at initial evaluation at our clinic were included. POTS was diagnosed based on at least six months of frequent debilitating symptoms of orthostatic intolerance, plus a consistent heart rate increase of at least 40 beats per minute without orthostatic hypotension on standing test. Patients with a Beighton score of at least 5/9 plus other systemic findings suggestive of EDS were further evaluated in Connective Tissue Disorders clinics. Results There were 362 patients meeting inclusion criteria, of which 82 patients had EDS (22.7%) and 141 patients had HSD (39.0%). Patients with EDS had an earlier median age at symptom onset (12.1 vs. 13.5 years, p=0.004) and longer median symptom duration (2.5 vs. 1.5 years, p=0.0008) compared to patients without hypermobility. Conclusions Our evaluation of a large series of pediatric patients with POTS revealed that over one-fifth of patients had EDS and over one-third of patients had HSD. The awareness of the prevalence of comorbidities such as hypermobility d…
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There is growing recognition of an association between autonomic dysfunction and hEDS/HSD.
“There is growing recognition of an association between autonomic dysfunction and hEDS/HSD and the group agreed it is an important comorbidity, commonly presenting as Postural Orthostatic Tachycardia Syndrome (POTS) in older children.”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [34]
Patients with hEDS/HSD in a pediatric POTS registry had longer median symptom duration (2.5 vs. 1.5 years, p = 0.0008) than patients without hypermobility.
“In a pediatric POTS registry, 62% of patients had hEDS/HSD (Beighton score ≥ 5/9), and earlier median age at symptom onset (12.1 vs. 13.5 years, p = 0.004) and longer median symptom duration (2.5 vs. 1.5 years, p = 0.0008) than patients without hypermobility [34].”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [34]
In a pediatric POTS registry, 62% of patients had hEDS/HSD (Beighton score ≥ 5/9).
“In a pediatric POTS registry, 62% of patients had hEDS/HSD (Beighton score ≥ 5/9), and earlier median age at symptom onset (12.1 vs. 13.5 years, p = 0.004) and longer median symptom duration (2.5 vs. 1.5 years, p = 0.0008) than patients without hypermobility [34].”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [34]
Patients with hEDS/HSD in a pediatric POTS registry had earlier median age at symptom onset (12.1 vs. 13.5 years, p = 0.004) than patients without hypermobility.
“In a pediatric POTS registry, 62% of patients had hEDS/HSD (Beighton score ≥ 5/9), and earlier median age at symptom onset (12.1 vs. 13.5 years, p = 0.004) and longer median symptom duration (2.5 vs. 1.5 years, p = 0.0008) than patients without hypermobility [34].”
From Pediatric joint hypermobility: a diagnostic framework and narrative review (2023) · cites this paper as [34]
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References 33 · Cited by 27
- Authors
- Boris, J. R., & Bernadzikowski, T.
- Affiliations
- Children's Hospital of Philadelphia (US)
- Corresponding author
- Bernadzikowski, Thomas
- ORCID
- Boris, Jeffrey R.
- Venue
- Autonomic Neuroscience
- Publisher
- Elsevier BV
- Published
- 2021-03
- Volume
- 231
- Pages
- 102770
- ISSN
- 1566-0702 (ISSN-L)
- OpenAlex
- W3116253346
- Cited by
- 27
- References
- 33
- Citations by year
- 2026: 5 · 2025: 5 · 2024: 5 · 2023: 6 · 2022: 4 · 2021: 2
- FWCI
- 2.74
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Autonomic Neuroscience (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Children's Hospital of Philadelphia
- Topics
- Health Sciences › Medicine › Surgery › Cardiovascular Syncope and Autonomic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Medicine › Psychiatry and Mental health › Fibromyalgia and Chronic Fatigue Syndrome Research
- Keywords
- postural orthostatic tachycardia syndrome, joint hypermobility, Ehlers-Danlos syndrome, hypermobility spectrum disorders, pediatric patients, prevalence, comorbidities, Beighton score, orthostatic intolerance, symptom onset age, symptom duration
- MeSH terms
- Adolescent, Adult, Child, Ehlers-Danlos Syndrome, Humans, Joint Instability, Prevalence, Orthostatic Intolerance, Postural Orthostatic Tachycardia Syndrome
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