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Diagnosis of platelet dysfunction in children: clinical predictors and test methods
Bhavya S Doshi1,2,3, Eric N Thompson4, Walter Faig5
1Division of Hematology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA.
Insights
Pediatric platelet function disorders (PFD) are more common in younger males. Whole-blood impedance lumiaggregometry (WBILA) effectively rules out PFD in children, showing high sensitivity and specificity.
Area of Science:
- Pediatric Hematology
- Hemostasis and Thrombosis
- Diagnostic Accuracy
Background:
- Platelet function disorder (PFD) diagnosis in children is challenging due to limited hemostatic exposure and large blood volume requirements for traditional tests like light transmission aggregometry (LTA).
- Limited data exists on the performance of whole-blood methods, such as whole-blood impedance lumiaggregometry (WBILA), for pediatric PFD evaluation.
Purpose of the Study:
- To identify clinical variables associated with a PFD diagnosis in pediatric patients.
- To assess the diagnostic performance of LTA and WBILA in children evaluated for PFD.
Main Methods:
- Retrospective cohort study of 667 children evaluated for PFD at a single center.
- Medical chart abstraction for demographics, medications, bleeding characteristics, and laboratory results.
- Univariate and multivariable analyses to determine associations between clinical variables and PFD diagnosis, comparing LTA and WBILA.
Main Results:
- 20.5% of children were diagnosed with PFD. Younger males had a higher incidence of PFD.
- Mucocutaneous bleeding and bleeding severity scores did not correlate with increased PFD odds.
- Both LTA and WBILA demonstrated high sensitivity (>91%) and specificity (>84%). Multivariable analysis identified younger age, male sex, thrombocytopenia, genetic disorders, and GI bleeding associated with PFD in the WBILA cohort.
Conclusions:
- Younger, male pediatric patients exhibit a higher incidence of PFD.
- Whole-blood impedance lumiaggregometry (WBILA) is a reliable method for ruling out PFD in children, demonstrating significant diagnostic accuracy.
Abstract:
Evaluation for platelet function disorders (PFD) in children is complicated by their limited exposure to hemostatic challenges, large volumes needed for light transmission aggregometry (LTA) testing, and limited data on the performance characteristics of whole-blood methods such as whole-blood impedance lumiaggregometry (WBILA). The objective of this study was to determine the clinical variables associated with the diagnosis of a PFD. A single-center, retrospective, cohort study of children evaluated for PFD was conducted. Medical charts were abstracted for demographics, medications, testing indications, bleeding sites and severity, and laboratory results. Univariate odds ratios (OR) and multivariable modeling were conducted for association of clinical variables with PFD diagnosis in children tested by LTA or WBILA. Of 667 patients, 20.5% were diagnosed with a PFD. The PFD cohort was more likely male (OR, 1.54; P = .025) and younger (8.8 vs 10.1 years; P = .026). Neither mucocutaneous bleeding (most common presenting indication) nor bleeding severity scores correlated with increased odds of PFD diagnosis. Both LTA and WBILA showed sensitivity of >91% and specificity of >84% for PFD diagnosis. A multivariable model identified younger age, male sex, thrombocytopenia, genetic disorders, and gastrointestinal bleeding with PFD diagnosis in the WBILA cohort. In conclusion, younger, male patients have a higher incidence of PFD. These data support that WBILA can effectively rule out PFD in a pediatric population.
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