Background: Epistaxis is a common childhood bleeding symptom, not only in children with an underlying inherited MBD, i.e., von Willebrand disease (VWD) or a platelet function disorder (PFD), but also in healthy children. An epistaxis-specific bleeding assessment tool (epistaxis-BAT) could be useful in screening for affected children. In addition to an indication of clinical severity, as determined by the Pediatric Bleeding Questionnaire (PBQ) or ISTH-BAT, an epistaxis-BAT should also include features of epistaxis that are predictors of a diagnosis of a MBD.

Aims: To determine which features of epistaxis are predictors of a diagnosis of a MBD in children.

Methods: Univariate and multiple logistic regression analyses were performed on previously collected data from the epistaxis section of the PBQ administered to children diagnosed with VWD or a PFD and healthy controls, < 18yrs (Stokhuijzen et al, J Pediatr 2018). The epistaxis features analyzed were: duration (>10min); frequency (>5/yr); spontaneous onset; site, specifically, occurring from both nostrils; and presence of seasonal correlation.

Results: 66 patients with a diagnosis of VWD or a PFD (median age: 12 yrs; 59% male) and 56 controls (median age: 12 yrs; 50% male) were included in the analysis. Using univariate logistic regression, the features of epistaxis that were found to increase the odds of a MBD diagnosis were: duration (odds ratio (OR) 16.7; 95% confidence interval (CI), 6.4-53.2); frequency (OR 8.2; 95% CI, 3.7-19.6); site (OR 7.7; 95% CI, 3.4-18.3); and seasonal correlation (OR 2.9; 95% CI, 1.3-6.6). Subsequent multiple logistic regression analysis showed that duration (OR 10.9; 95% CI, 3.2-46.8) and frequency (OR 4.36; 95% CI, 1.6-12.8) were the major predictors for an underlying MBD.

Conclusions: Duration and frequency of epistaxis are the strongest predictors of a diagnosis of a MBD in children. Thus, these features would be important to include in an epistaxis-BAT.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/features-of-epistaxis-predictive-of-a-mucocutaneous-bleeding-disorder-mbd-in-children/