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Desmopressin responsiveness in children with Ehlers‐Danlos syndrome associated bleeding symptoms
Author(s) -
Mast Kelley J.,
Nunes Mark E.,
Ruymann Frederick B.,
Kerlin Bryce A.
Publication year - 2009
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2008.07446.x
Subject(s) - desmopressin , medicine , ehlers–danlos syndrome , retrospective cohort study , pediatrics , cohort , surgery
Summary 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.