• Monika Kozyra, Regina Kostyun, and Sara Strecker.
• Bone and Joint Institute, Hartford Hospital, Hartford, CT.
• Medicine (Baltimore). 2024 Oct 11; 103 (41): e39212e39212.

AbstractClinical features of hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility syndrome (HMS) have classically focused on dysfunctions related to the musculoskeletal system. A growing body of literature suggests substantial multisystemic involvement, although this has not been recapitulated in a pediatric/young adult population. Leveraging a large United States healthcare claim database illuminates multisystem disorders among patients diagnosed with hEDS and HMS in the age range of 10 to 24. This was a retrospective review of patient records within the de-identified healthcare claims database, PearlDiver. Patients with a diagnosis of hEDS or HMS, and those without these diagnoses who were seen for their annual physical examination, between the ages of 10 and 24, were queried for the presence of additional medical conditions. Descriptive statistics were used to define the frequency of multisystem diagnoses. Nineteen thousand seven hundred ninety hEDS patients, 17,509 HMS patients, and 4,959,713 patients from the general population were analyzed. Within 2 years following hEDS or HMS diagnosis, digestive disorders were the most prevalent diagnosis, followed by cardiovascular conditions. Digestive disorders occurred in 54.6% of patients with hEDS and 41.6% of patients with HMS, compared to 28.5% of the general population. Cardiovascular disorders occurred in 43.6% of patients with hEDS and 21.8% of patients with HMS compared to 10.3% of the general population. Anxiety, respiratory disorders, and developmental disorders occurred in approximately 25% of the hEDS group and 20% of the HMS group, compared to ~15% of the general population, all statistically significantly higher in the hEDS and HMS groups. This study highlights multisystem diagnoses within the pediatric hEDS/HMS populations. hEDS patients had higher rates of multisystem diagnoses compared to HMS patients. These results suggest a high multisystem disease burden for young hEDS/HMS patients. Future research is needed to understand the timing and presentation of clinical symptoms for this population.Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.

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