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

Kelley J Mast1, Mark E Nunes, Frederick B Ruymann

  • 1Department of Laboratory Medicine, Nationwide Children's Hospital, Columbus, OH 43205-2696, USA.

British Journal of Haematology
|November 28, 2008
PubMed

Insights

Desmopressin therapy effectively reduced bleeding times in children diagnosed with Ehlers-Danlos Syndrome (EDS), a condition linked to collagen defects. This study indicates desmopressin normalizes bleeding in pediatric EDS patients.

Area of Science:

  • Medical research
  • Hematology
  • Genetics

Background:

  • Ehlers-Danlos Syndrome (EDS) involves heritable collagen defects.
  • Bleeding symptoms are a potential complication of EDS.
  • Desmopressin has shown promise in case reports for reducing bleeding in EDS patients.

Purpose of the Study:

  • To evaluate desmopressin's effectiveness in managing bleeding times in pediatric EDS patients.
  • To assess the responsiveness of EDS-associated bleeding manifestations to desmopressin treatment.

Main Methods:

  • Retrospective chart review of pediatric patients with EDS and bleeding symptoms.
  • Analysis of bleeding times before and after desmopressin administration.
  • Inclusion of 26 children, with 19 undergoing a desmopressin challenge.

Main Results:

  • The mean bleeding time decreased significantly from 11.26 minutes to 5.95 minutes post-desmopressin treatment (P < 0.01).
  • A substantial majority (73%) of the included children received desmopressin therapy.
  • The results demonstrate a clear reduction in bleeding times with desmopressin.

Conclusions:

  • Desmopressin therapy is effective in normalizing bleeding times for children with Ehlers-Danlos Syndrome.
  • This finding supports the use of desmopressin in managing bleeding manifestations associated with EDS in pediatric populations.

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