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Glanzmann thrombasthenia (GT) is an inherited genetic disorder affecting platelets, which is characterized by spontaneous mucocutaneous bleeding and abnormally prolonged bleeding in response to injury or trauma. The underlying defect is failure of platelet aggregation due to qualitative and/or quantitative deficiency of platelet integrin αIIbβ3 resulting(More)
OBJECTIVE To determine the frequency and clinical features of bleeding disorders in the tribe as a result of consanguineous marriages. DESIGN Cross Sectional Study INTRODUCTION Countries in which consanguinity is a normal practice, these rare autosomal recessive disorders run in close families and tribes. Here we describe a family, living in village Ali(More)
OBJECTIVE To determine the frequency of inherited bleeding disorders, its complications, and treatment modalities available for its treatment. DESIGN Cross-sectional study. PATIENTS AND METHODS Patients with a history of bleeding tendency were tested for confirmation of the diagnosis. History and clinical findings were recorded. Laboratory analysis(More)
The aim of the study was to assess the prevalence of HCV, HBV, and HIV infections among the patients with hemophilia. Patients with Hemophilia A and B were evaluated who visited hospital for factor replacement therapy. The viral markers tested in these patients included anti-HCV-Ab, HBsAg, and anti-HIV-Ab. Seroprevalence was compared from 5717 exchange(More)
This study was conducted on patients with a history of congenital bleeding disorders or with suspected bleeding tendencies. Laboratory analysis revealed Von Willebrand disease (VWD) in 68 (21.3%) of 318 participants with male to female ratio of 0.8: 1 (31 to 37) and median age 17 years (range 2-45 years). Type 3 being the most frequent, 35 (51.4%) of 68,(More)
OBJECTIVE To determine the frequency of factor VIII specific inhibitors in haemophilia A. STUDY DESIGN Cross-sectional study. PLACE AND DURATION OF STUDY National Institute of Blood Disease and Bone Marrow Transplantation, Karachi, from August 2007 to March 2009. METHODOLOGY Venous blood samples of diagnosed haemophilia A patients were collected in(More)
OBJECTIVE The aim of the study was to determine the frequency and reasons for donor deferral prior to the blood donation process in our population. BACKGROUND Transfusion is an irreversible event that carries potential risks as well as benefits to the recipient. Therefore, donor selection prior to blood donation is one of the most important steps in(More)
Fibrinogen is a plasma glycoprotein mainly synthesised by hepatocytes and circulating as a 340-kDa hexamer consisting of two sets of three different polypeptide chains (Aα, Bβ, and γ, encoded by the FGA, FGB, and FGG gene, respectively). Congenital afibrinogenaemia and hypofibrinogenaemia are rare bleeding disorders characterised by abnormally low levels of(More)
BACKGROUND In Pakistan routine blood group typing of thalassemia patients identifies ABO and Rh(D) antigens only. Therefore, other antigen incompatibilities between blood donor and blood recipient may cause alloimmunisation. OBJECTIVE The aim of this study was to estimate the frequency of alloimmunisation and to evaluate the risk factors associated with(More)