The International Society on Thrombosis and Haemostasis Bleeding Assessment Tool's Usefulness as a Screening Questionnaire for Diagnosing and Assessing the Severity of Glanzmann Thrombasthenia in Children from Northeast Egypt

国际血栓与止血学会出血评估工具作为筛查问卷在埃及东北部儿童格朗茨曼血小板无力症诊断和严重程度评估中的应用价值

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Abstract

OBJECTIVES: to assess the utility of the international society on thrombosis and hemostasis (ISTH) bleeding assessment tool a screening questionnaire for diagnosis of Glanzmann thrombasthenia (GT) and its severity. SUBJECTS &METHODS: Out of 250 children, only 30 had GT and were classified into 3 sub-groups by flow cytometric analysis. Thirty age- and sex-matched healthy individuals were compared to the cases. Platelet count, bleeding time, platelet aggregation studies, and flow cytometric analysis were done. RESULTS: Dental extractions, oral cavity bleeding, bruises on the skin, bleeding from small cuts, and epistaxis all exhibited significant increases in the cases as compared to the healthy individuals (p = < 0.001). In comparison to type II and type III, the ISTH-bleeding questionnaire score revealed a significant rise in the type I (p < 0.001). The ISTH-bleeding questionnaire score's AUC revealed that GT cases and healthy individuals could be distinguished with great accuracy (AUC = 1) (CI 95%, P < 0.001). The following values were 100% at the cutoff value of ≥ 3.5: sensitivity, specificity and accuracy. CONCLUSION: It will be helpful to screen cases for suspected bleeding disorders, such as GT using the ISTH bleeding assessment tool questionnaire before undergoing time-consuming examinations. This will also help forecast the severity of various types of GT. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12288-025-01994-0.

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