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[Is the DDAVP-test helpful to diagnose children?]
E Weissenbacher1, B Acham-Roschitz, B Leschnik
1Medical University of Graz, Auenbruggerplatz 30, A-8036 Graz, Austria. wolfgang.muntean@meduni-graz.at
Insights
The DDAVP test is not a reliable diagnostic tool for identifying mild von Willebrand disease (VWD) in children with borderline VWF parameters. A positive response to DDAVP does not significantly correlate with bleeding history in diagnosing VWD.
Area of Science:
- Hematology
- Pediatric Medicine
- Diagnostic Testing
Context:
- Diagnosing von Willebrand disease (VWD) in pediatric patients with borderline VWF parameters presents challenges.
- The 'grey area' of VWD makes it difficult to distinguish mild disease from low-normal VWF levels, especially given difficulties in assessing children's bleeding history.
Purpose:
- To evaluate the efficacy of the 1-deamino-8-D-arginine vasopressin (DDAVP) test as a diagnostic tool for VWD in pediatric patients with borderline VWF parameters.
- To determine if DDAVP response correlates with bleeding history in this patient group.
Summary:
- A retrospective analysis of 52 pediatric patients with borderline VWD evaluated the DDAVP test's diagnostic utility.
- While all patients showed a good response to DDAVP, the increase in VWF:Ag, VWF:RiCof, and FVIII:C did not significantly differ between patients with positive and negative bleeding histories.
- The study found no significant association between a positive bleeding history and a lower DDAVP response, nor between a high response and a negative history.
Impact:
- The DDAVP test is not a suitable diagnostic tool for the 'grey area' of VWD in pediatric patients.
- This finding highlights the need for alternative diagnostic strategies for VWD in children with borderline VWF levels.
Unlabelled:
It is very difficult to determine if patients with a moderate low level of VWF parameters have mild disease or if they are just low normal (so called grey area of VWD). This applies particularly to pediatrics, because it is difficult to evaluate the bleeding history of children. Al our centres every child diagnosed with vWD gets DDAVP to test the response for it. This study was done to evaluate the DDAVP- test as a diagnostic tool.
Patients, Methods:
A retrospective analysis of data obtained with routine DDAVP administration for test purposes in 52 patients with borderline von Willebrand disease at the haemophilia centre Graz was done. The increase of VWF:Ag, VWF:RiCof and FVIII:C has been document and compared.
Results:
All of our patients had a very good response after application of DDAVP. The increase of VWF:Ag, VWF:RiCof and FVIII:C was compared in patients with positive and negative bleeding anamneses. The patients with positive anamneses had significantly lower parameters at the beginning. The increase of VWF parameters did not differ significantly between the groups at the different time-points. These results demonstrate that a positive anamnesis is not significantly associated with a lower increase. On the other side a high increase is not associated with a negative anamnesis.
Conclusion:
It is not possible to use the DDAVP test as a diagnostic tool for patients within the diagnostic grey area of VWD.
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