Glanzmann’s Thrombasthenia: Epistaxis Treatment and Outcomes in Children

Alblowi, Thikra Mohammed; Alshammari, Fahad Nashmi; Alshammari, Bashayer Aqeel; Elazab, Ahmed Ali; Albilasi, Thamer Menwer; Alotaibi, Hind Jaza

doi:10.21608/ejhm.2018.10884

Glanzmann’s Thrombasthenia: Epistaxis Treatment and Outcomes in Children

Document Type : Original Article

Authors

Hail University, Collage of Medicine

10.21608/ejhm.2018.10884

Abstract

Background: Glanzmann’s Thrombasthenia (GT) is a rare inherited blood clotting (coagulation) disorder characterized by the impaired function of specialized cells (platelets) which are essential for proper blood clotting. Objectives: The present study aimed to assess the management steps of epistaxis in children with GM disease and assess the clinical outcome and the success rate or field of management.
Methods: Data was taken via scanning all the patients’ files in ENT clinic and 4 children with GM were identified as receiving therapy. Furthermore, data collected from each file were primary complaint needing a duration of hospitalization, intensive care unit (ICU) admission, coagulation management, hematologic results, medical therapy administration, blood products transfusion and the surgical intervention performed.
Results: With a total of 25 hospital presentations, the highest percentage of the present symptoms was for epistaxis with 68% ⁽¹⁷⁾, then 16% ⁽⁴⁾ for gingival bleeding and 8% for each of menorrhagia and hematemesis. Conclusion and recommendation: Our study carried out the most effective technique that control epistaxis is Fresh Frozen (FFP) Plasma. The epistaxis in GT should be concern as sever threating life condition and should be managed with effective treatment.

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