Abstract

To compare bone parameters between individuals with hypermobile Ehlers-Danlos syndrome (hEDS) and generalized joint hypermobility spectrum disorder (G-HSD), both diagnosed according to the most recent diagnostic criteria, and with controls. Twenty female adults with hEDS (mean age 43.8years), 20 with G-HSD (mean age 41.8years), and 37 healthy controls (mean age 40.8years) participated. Body composition and bone parameters at whole body and lumbar spine were assessed by dual-energy X-ray absorptiometry. Peripheral quantitative computed tomography at the lower leg evaluated body composition (66 % site), and trabecular (4% site) and cortical (66% site) bone parameters at the tibia. No significantly different body composition and bone parameters were observed between hEDS and G-HSD. Compared to controls, individuals with hEDS and G-HSD had lower muscle mass (p=0.004 and p<0.001, respectively) and cross-sectional area (p=0.025 and p<0.001, respectively), cortical bone mineral content (BMC; p=0.024 and p=0.027, respectively) and area (p=0.019 and p=0.010, respectively). Additionally, individuals with hEDS had lower muscle density (p=0.009), trabecular BMC (p=0.027) and bone mineral density (p=0.022), and individuals with G-HSD lower stress-strain index (p=0.019), and periosteal and endosteal circumference (p=0.002 and 0.025, respectively), compared to controls. Results indicated lower cortical bone mineral content and smaller cortices in hEDS and G-HSD compared to controls. Individuals with hEDS and G-HSD had no different bone parameters, suggesting that these impairments might not be reflected by the different diagnostic classification. Therefore, we recommend regular physical activity, and training to reduce the risk of falling in both hEDS or G-HSD.

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