Author(s):
1. Nazish Saqlain:
Department of Hematology and Transfusion Medicine, The Children’s Hospital & Institute of Child Health, Lahore, Pakistan.
2. Tooba Fateen:
Department of Hematology and Transfusion Medicine, The Children’s Hospital & Institute of Child Health, Lahore, Pakistan.
3. Hammad Tufail:
Department of Hematology , Taif University, Taif KSA.
4. Naghmana Mazher:
Department of Pathology, Fatima Jinnah Medical University, Lahore, Pakistan.
Abstract:
Objectives: To assess the utility of ISTH-BAT (International Society on Thrombosis and Hemostasis- Bleeding Assessment Tool) in the diagnosis of Glanzmann Thrombasthenia (GT) in comparison to controls. Methods: It was a case-control study carried out at The Children's Hospital, Lahore from January 2012 to May 2021. All patients from neonates to 18 years with a final diagnosis of GT were studied retrospectively. The clinical details were collected from hospital records and telephonically on ISTH-BAT questionnaire after taking informed consent. The same proforma was obtained from 75 healthy controls. Data was analyzed on SPSS version 26. Results: Out of 427 patients with suspected platelet function disorders, 133 were diagnosed as GT. The mean age was 7.29±5 years. Male to female ratio was 1.1:1. Among cases, 76.6% were products of consanguineous marriage. Epistaxis was the commonest symptom with highest score (p value < 0.001). Cutaneous and oral cavity bleeds were more severe and frequent in patients than controls (p value < 0.004). The median ISTHBAT score among patients was nine while in control group was one. Sensitivity was 86.4%, specificity was 77.3%, positive predictive value was 0.87 and negative predictive value was 0.76. Area under the receiver operator curve was 0.78 (95% confidence interval 0.82-0.90, p < 0.001*). Conclusion: ISTH-BAT scores were significantly higher in GT patients than controls. So, we recommend the inclusion of ISTH-BAT in diagnostic evaluation of patients with suspected Glanzmann Thrombasthenia.
Page(s):
791-795
DOI:
DOI not available
Published:
Journal: Pakistan Journal of Medical Sciences, Volume: 38, Issue: 4, Year: 2022
Keywords:
Children
,
Questionnaire
,
bleeding
,
Children
,
Glanzmann Thrombasthenia
,
Platelet function disorders
References:
References are not available for this document.
Citations
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