Literature DB >> 19036109

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

Kelley J Mast1, Mark E Nunes, Frederick B Ruymann, Bryce A Kerlin.   

Abstract

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.

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Year:  2008        PMID： 19036109     DOI： 10.1111/j.1365-2141.2008.07446.x

Source DB:  PubMed          Journal:  Br J Haematol        ISSN： 0007-1048            Impact factor:   6.998


  9 in total

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