University of Khartoum

Detection of Bleeding Disorders in Sudan with Special Reference to von Willebrand Disease

Detection of Bleeding Disorders in Sudan with Special Reference to von Willebrand Disease

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Title: Detection of Bleeding Disorders in Sudan with Special Reference to von Willebrand Disease
Author: Elabbas, Imtithal Elsayed Ibrahim
Abstract: Background: von Willebrand disease (vWD) is the commonest inherited bleeding disorder caused by deficiency or dysfunction of von Willebrand factor.The aim of this study was to look for undiagnosed patients with possible bleeding disorders with special reference to vWD and to offer them the necessary investigations. Also to evaluate the clinical utility of a bleeding scoring system for vWD. Methods: This was a descriptive cross-sectional hospital based study conducted in the Hemophilia centre, Khartoum Teaching Hospital from March 2014 to July 2015. The plan of this outreach project was based on searching for new patients, using two approaches. First, awareness presentations were done to clinicians to identify patients with possible bleeding disorders and refer them to the haemophilia centre for assessment. Second was to contact the registered vWD patients to send their family members with bleeding symptoms to be assessed. The referred patients were then assessed by a numerical scoring system which gives scores to bleeding symptoms according to severity. They were also asked about a family history of a bleeding tendency. Those with a score of 5 or more and/ or had a positive family history were investigated. The patients, were investigated by taking blood samples and doing CBC, PT, APTT, finger prick screening test for platelet aggregation, platelet aggregometry, coagulation factors assay, vWF:Ag assay and ABO blood grouping. The study included total of 107 patients. The data were collected via a questionnaire designed for this purpose and analyzed using Statistical package for social science program. Results : Out of 107 patients investigated 72 were males (67.2%), 74 patients of whom were below 16 years, 22 (20.6%) were found to have vWD, 20 cases (18.6%) had platelet dysfunction, 20 cases (18.6%) had haemophilia A, 14 cases (13%) had haemophilia B (one being a female), 15 cases with other inherited clotting factors deficiencies. Two carriers were identified, one a hemophilia A and one a hemophilia B carrier, 14 of the investigated patients (13%) showed no coagulation abnormality. Of the 22 patients with vWD, the majority were males below 16 years, 15 (68.2%) had a positive family history of bleeding, 4 (18.2%) had type 3 vWD, 18 (81.8%) patients had type 1 vWD, 8 (36.4%) had bleeding score < 5 (normal). In this study, the bleeding score had a sensitivity of 63.6% for diagnosis of vWD but is not specific for it. APTT < 36 (normal) was found in 7(33.3%) patients and 3(13.6%) patients had normal FVIII level. Conclusion : This outreach project was effective in reaching for and diagnosing a good number of patients with vWD and other bleeding disorders. The bleeding score is a valuable tool in the initial assessment of bleeders. Minor bleeding symptoms and normal screening coagulation investigations do not exclude vWD.
URI: http://khartoumspace.uofk.edu/123456789/25018


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