الفهرس | Only 14 pages are availabe for public view |
Abstract Bleeding diathesis is caused by quantitative and qualitative alterations of either platelets or plasma proteins involved in coagulation and fibrinolysis. The severity is generally related to the degree of the underlying defect. Rapid and reliable identification of these diseases is important to allow the adoption of appropriate substitutive or supportive therapies. The study was formulated on the observation that in Egypt, genetic diseases do not receive any public health support because they are considered to be rare conditions with low prevalence. This is an observational descriptive study in Sohag university hospital in period from 1st of May 2013 to 30th of April 2014 on children from one month to 12 years old who were suspected to have bleeding diathesis careful history taking, clinical examination, laboratory investigations (CBC, bleeding time, PT, PC, aPTT, liver function tests, BM aspiration, factor assay and platelet function tests) and abdominal ultrasound. Our study included the following bleeding disorders; ITP, Aplastic anaemia, Glanzman thrombasthenia, Wiskot Aldrish syndrome, Leukemia, Von willbrand disease, Hemophilia A, Hemophilia B, Hypofibrinogenemia, Factor VII deficiency, Late vitamin K deficiency, Liver disease, DIC (disseminated intravascular coagulation), Non Hodgking lymphoma and Hodgking lymphoma. We performed a clinical and laboratory analysis of 130 children with bleeding disorders and founded that ITP was the commonest bleeding disorders in our locality followed by Hemophilia A and the most symptoms presented by children was ecchymosis followed by purpura, epistaxis and bleeding gums and CBC, PT, PC, aPTT were the most valuable tools in diagnosis. |