Desmopressin responsiveness in children with Ehlers-Danlos syndrome associated bleeding symptoms

Ehlers-Danlos Syndrome (EDS) is caused by heritable collagen defects and may be associated with bleeding symptoms. Desmopressin has been described in case reports to decrease bleeding times in these patients. This study sought to assess bleeding time responsiveness to desmopressin therapy in a cohort of children with EDS-associated bleeding manifestations. A retrospective chart review of children with EDS referred for bleeding symptoms was utilized. Twenty-six children were included; 19 (73%) had a desmopressin challenge. The mean bleeding time was 11.26 (+/- 4.39) min, decreasing to 5.95 (+/- 2.41) min with treatment (P < 0.01). Desmopressin normalizes bleeding times in children with EDS.