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Browsing Dissertations by Author "AWUKU, NANA AGYEIWAH"

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    FACTOR VIII INHIBITOR STATUS OF HAEMOPHILIA A PATIENTS AT THE KORLE-BU TEACHING HOSPITAL
    (Ghana College of Physicians and Surgeons, 2023-03-31) AWUKU, NANA AGYEIWAH
    Background: Haemophilia is an inherited bleeding disorder resulting from mutations in the Factor VIII (FVIII), Factor IX (FIX) or Factor XI (FXI) gene. These mutations give rise to Haemophilia A, B or C respectively. Haemophilia A and B clinically manifest as bleeding into joints, soft tissues, and muscles spontaneously or after minor trauma whilst Haemophilia C is commonly associated with mucosal bleeding which is usually provoked. Recombinant factor replacement is the mainstay of treatment for Haemophilia A, with inhibitor formation remaining the major challenge in the treatment with factor concentrate. General Aim: This study determined the factor VIII inhibitor status among patients with Haemophilia A at the Korle-Bu Teaching Hospital Methods: Cross-sectional study involving all haemophilia A patients attending both adult and paediatric clinics at Korle-Bu Teaching Hospital (KBTH). Patients were recruited after giving informed consent or assent and or parental permission where appropriate. A data extraction form was used to extract clinical data from the patient’s medical records/folder.4.5mls of blood was taken for measuring residual FVIII activity and inhibitor assay using Bethesda Assay. Statistical analysis was done using STATA version 14. Results: All eighty-one (81) participants were male with 70.4%, 14.8%, 14.8% having severe, moderate, and mild disease respectively. The mean age of participants was 17.1 years (±13.5). Majority (93.8%) of participants had received some form of therapy which included recombinant FVIII concentrate, plasma derived FVIII concentrate, cryoprecipitate and emicizumab. A small number (6.2%) of participants had never been exposed to any form of therapy. Prevalence of inhibitors in this study was found to be 11.1% with 85.7% (n=6) having low titre inhibitors and 14.3% (n=1) having high titre inhibitors. All inhibitor positive participants were on on-demand therapy with majority (85.7%) having severe disease. Inhibitor positive participants had had higher bleeding episodes in the past year 2.14 (p=0.286). Most (57.1%) of the inhibitor positive participants had received only recombinant factor concentrate and 42.9% had received both recombinant and plasma derived concentrates (p=0.211). xiv Bleeding from the oral cavity was the commonest presentation at diagnosis (32.1%) followed by joint swelling and pain (21.0%). Almost half (46.9%) presented with spontaneous bleeding at diagnosis and 53.1% had provoked bleeding out of which 60% was trauma related and 40% from surgical/medical procedure. A small percentage (6.2%) of participants were on prophylactic therapy. Conclusion: This study reported prevalence of FVIII inhibitor in haemophilia A patients to be 11.1% with majority of participants classified as severe haemophilia A. There is the need for education and increased awareness for early diagnosis and prevention of complications of haemophilia A; especially the development of inhibitors in Ghana to enable institution of appropriate therapy.