Congenital thrombasthenia in children welfare teaching hospital : a descriptive study

الملخص EN

Background: Thrombasthenia is an inherited genetic disorder affecting platelets, which is characterized by spontaneous muco-cutaneous bleeding and abnormally prolonged bleeding in response to injury or trauma.

Objectives: The aim of this study was to assess the diagnosis and treatment of thrombasthenia in Children Welfare Teaching Hospital.

Type of the study: A cross-sectional study.

Methods: This descriptive study was performed on 66 patients with thrombasthenia from the first of October 2013 till the first of July 2015.The diagnosis of the disease was done by a wide spectrum of characteristics including family history, clinical manifestations, laboratory tests..

Results: The common manifestations of the disease at time of diagnosis were epistaxis in 23 patients (34.8%) and gum bleeding in 22 patients (33.3%) .Mostly established at age 1-10years.Family history was positive in most patients.hepatitis C was rare in our patients after platelet transfutions.

Conclusions: : It is a rare hemorrhagic disease in our center, mostly presented as minor bleeding involving mainly mucocutaneous regions which appears at an early childhood with positive family history in most cases.