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

Br J Haematol 2009 Jan 19;144(2):230-3. Epub 2008 Nov 19.

Department of Laboratory Medicine, Nationwide Children's Hospital, Columbus, OH 43205-2696, USA.

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.

Download full-text PDF

Source
http://dx.doi.org/10.1111/j.1365-2141.2008.07446.xDOI Listing
January 2009

Publication Analysis

Top Keywords

bleeding symptoms
12
bleeding
8
associated bleeding
8
ehlers-danlos syndrome
8
children eds
8
bleeding times
8
bleeding time
8
children
5
desmopressin
5
retrospective chart
4
chart review
4
review children
4
manifestations retrospective
4
referred bleeding
4
eds referred
4
assess bleeding
4
symptoms utilized
4
eds-associated bleeding
4
therapy cohort
4
responsiveness desmopressin
4

Similar Publications