1. SIGNS AND SYMPTOMS OF HYPERMOBILITY EHLERS-DANLOS SYNDROME (hEDS) TO CONSIDER WHEN DIAGNOSING THIS CONDITION
Jaime F. Bravo1, 2, Carlos Wolff 3
1.Clínica Arauco Salud 2. Servicio Reumatología, Hospital San Juan de Dios.
3.- Depto. Medicina Occidente, Universidad de Chile
Objective. Increase awareness of Hypermobility Ehlers-Danlos Syndrome (hEDS), formerly known as Joint Hypermobility Syndrome (JHS), which usually goes undiagnosed.
Patients. A group of 1395 hEDS patients were studied in our adult Rheumatology Clinic, in Santiago, Chile. The Beighton score (BSc) and the Brighton Criteria (BC) were applied to all patients. A checklist was used to look for frequent signs and symptoms of hEDS. Not all questions or signs were checked in all patients. Age range: y/o. Average age: Females 78.1%. Chi squared test used for statistical analysis.
Results. In the total studied group: Negative BSc: 56.9%.
Frequency of the signs studied are shown in Figure 1
Figure 1
Conclusions: The following signs and symptoms were frequent enough (≥ 45%) to feel that they are important when considering the diagnosis of hEDS: Hand holding the head; elephant paw; light blue sclera; horizontal thumb: valgus elbow; soft skin; flying bird hand; typical hEDS face; low BMD and Dys. There was no significant difference between sexes regarding low BMD at all ages. Dys was significantly more noticeable in women than in men at all ages. By definition the hEDS diagnosis implies a positive BC, thus in this study the BC was positive in 100% of the cases. As noted in our previous study, the BSc was again negative in 56.9% of the studied patients. This is important since in some studies, many hEDS patients are not included when only applying the BSc rather than the BC. We suggest preparing a checklist similar to the one used when diagnosing hEDS.
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