University of Khartoum

Congenital Platelet Dysfunction in Haemophilia Center in Khartoum Teaching Hospital

Congenital Platelet Dysfunction in Haemophilia Center in Khartoum Teaching Hospital

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Title: Congenital Platelet Dysfunction in Haemophilia Center in Khartoum Teaching Hospital
Author: Ali, Lana Mohammed Abd Elmotalib
Abstract: Background: Congenital platelet dysfunction is a rare disease with significant morbidity and mortality. No studies to characterize this group of disorders were done in Sudan.This study was done in Haemophilia Center and underling laboratory in Khartoum Teaching Hospital. This is the only center in Sudan looking after patients with bleeding disorders including those with platelet dysfunction. Patients are offered diagnostic service, treatment and follow up. The main objective of the study was to characterize congenital platelet dysfunction in Sudan regarding the symptoms, investigations, classification and treatment. Also to compare between clot retraction and finger prick platelet aggregation tests and the role of each test in the diagnosis of this disorders. Methods: This was a descriptive case control study. Seventy one patients who were registered with hereditary platelet dysfunction were included. Data were collected using a predesigned questionnaire with clinical data, investigations, classification, and treatment. Clot retraction and finger prick platelet aggregation test were done for 30 patients with platelet dysfunction and 30 normal subjects. Thirty control subjects without bleeding disorders and were not on any drugs that affect platelet function. Results: Age of first presentation was more than 10 years in 42% of the patients. Epistaxis was present in 36.6%, menorrhagia in 36.8% in females, family history of the disease in 47.5% and anaemia in 21.2% of patients. Of the 71 patients, 14% were further classified as Glanzman`s thrombasthenia and 10% as Bernard Soulier syndrome while the rest, 76%, were diagnosed as platelet dysfunction but were not further classified. Treatment by tranexamic acid was used in 46.4% of patients, platelet transfusions were received by 28.1% and blood transfusions by 25.5 %. Regarding the clot retraction and finger prick platelet aggregation tests, thirty normal subjects had normal clot retraction and normal finger prick platelet aggregation. Clot retraction was poor in 29 patients and normal in one patient. Finger prick platelet aggregation test was normal in 3 patients and poor in 27 patients. Conclusions: Finger prick platelet aggregation test detect most of the patients with this disorder. Clot retraction test support the diagnosis of disease but cannot be used solely.
URI: http://khartoumspace.uofk.edu/123456789/25843


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