The prevalence of orthostatic hypotension, orthostatic intolerance and postural orthostatic tachycardia syndrome in Ehlers-Danlos hypermobility syndrome in children and adolescents

Hypermobile Ehlers-Danlos syndrome (hEDS) is a connective tissue disorder frequently associated with autonomic dysfunction. This study investigates the prevalence and characteristics of orthostatic disorders, including orthostatic hypotension (OH), orthostatic intolerance (OI), and postural orthostatic tachycardia syndrome (POTS), in paediatrics patients with hEDS. The objective of this study was to evaluate the prevalence and clinical features of autonomic disorders in children and adolescents with hEDS and explore associations with sex, age, and comorbid conditions. In this cross-sectional study, ۶۵ children and adolescents aged ۱۰–۱۹ years with confirmed hEDS were evaluated for autonomic dysfunction using standardized orthostatic testing. Demographic, clinical, and comorbidity data were collected and analysed. Orthostatic intolerance was the most common autonomic disorder (۷۶.۳%), followed by OH (۳۱.۶%) and POTS (۲۸.۹%). Female patients were more frequently affected than males (P < ۰.۰۵). Comorbidities, including chronic pain, fatigue, and gastrointestinal symptoms, were strongly associated with autonomic symptoms. Autonomic dysfunction is highly prevalent in paediatrics hEDS, particularly among female patients. Early recognition and multidiscipline management are essential to improve long-term outcomes.

کلیدواژه ها:

Ehlers-Danlos syndrome ، Hypermobility ، orthostatic intolerance ، POTS ، dysautonomia ، Paediatrics