publication . Article . 2017

the glanzmann s thrombasthenia in tunisia a cohort study

Hejer Elmahmoudi; Meriem Achour; Nejla Belhedi; Hend Ben Neji; Kaouther Zahra; Balkis Meddeb; Emna Gouider;
Open Access
  • Published: 20 Jul 2017 Journal: Journal of Hematology, volume 6, pages 44-48 (issn: 1927-1212, eissn: 1927-1220, Copyright policy)
  • Publisher: Elmer Press, Inc.
Abstract
Background: The Glanzmann’s thrombasthenia (GT) is a rare autosomal-recessive bleeding disorder with uncommon neonatal revelation. It is due to abnormalities of quantitative and/or qualitative α IIb β 3 integrin. This cell adhesion receptor is essential for platelet aggregation and allows the formation of a hemostatic plug if the vessel is damaged by injury. The clinical picture of GT is variable, with mucocutaneous bleeding due to non-functional platelets. Management requires a good expertise in bleeding disorders. We describe the clinical and the epidemiological data of GT in Aziza Othmana Hospital Hemophilia Center. Methods: This was a retrospective study of ...
Subjects
free text keywords: Cohort study, Platelet transfusion, Packed red blood cells, Retrospective cohort study, Population, education.field_of_study, education, Medicine, business.industry, business, Tranexamic acid, medicine.drug, Thrombasthenia, Pediatrics, medicine.medical_specialty, Glanzmann's thrombasthenia, medicine.disease
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