الفهرس 
COAGULATION DISorderSOnly 14 pages are availabe for public view
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Abstract
A common misperception exist that females do not have bleeding disorder .However, we now know this is far from true. Hemophiliac are most commonly x-linked recessive diseases characterized by deficiency of factor V111 (hemophilia A) or factor ix (hemophilia B, or christmas disease). The clinical severity correlates well with factor levels, and they are clinically classified as mild (>5%of normal factor activity), moderate (1%to 5%factor activity), and severe (<1%factor activity).Co-inheritance of the factor V Leiden mutation occurs in 5% of patients and results in a decreased bleeding tendency. The incidence is 1 per 5,000live births for hemophilia A and 1 per 30,000 live births for hemophilia B. In 30%of patients, hemophilia is the results of a de novo mutation, and no family history can be elicited. Males are most commonly affected: however, symptomatic females have been documented, and the proposed mechanisms include X-chromosome inactivation or deletion, or the presence of a true homozygous offspring of affected male and a carrier female.
Von will brand disease (VWd) constitutes a heterogeneous group of entities transmitted as an autosomal dominant or autosomal recessive trait depending on the variant type of the disease. The prevalence of VWd in the general population is much higher than estimated in the past ,it may be as high as 1% .The clinical presentation of the disease is variable ,mostly depending on the level of von Willebrand factor(VWF), including easy bruising ,expistaxis ,gastrointestinal ,post-operative ,post-traumatic hemorrhage ,menorrhagia and post-partum bleeding.
Von willebrand disease is far more common and is the commonest inherited bleeding abnormality affecting adolescents with a prevalence of .8% to 1.3% Von willebrand diseases the result of quantitative and /or qualitative defects of Von willebrand factor (vWF). Von willebrand factor servers as a carrier for FVIII in the circulation blood preventing it from inactivation; it also mediates platelet adhesion and thrombus formation at the sites of the sites of vascular injury.
This study was a retrospective and prospective study conducted on 374 female patients their age ranged from (birth to 18 years) they were collected from Benha university hospital, Benha teaching hospital, Friends institute of bleeding patients in El Helal El Ahamar, El-Mansoura health insurance hospital and from out patient clinics in Benha during the period from August, 2007 to August, 2009.
Coagulation disorders total number was 374 cases, the number and percentage of cases with Von Willebrand’s disease was 187 which represent (50%) of whole cases of bleeding disorders, the number of cases with Hemophilia A was 132 which represent (35.3%), the number of cases with Hemophilia B was 29 which represent (7.8%).
The number of cases with Hereditary deficiency of other clotting factors was 26 which represent (6.9%) classified as follows:
1. 6 cases with factor V deficiency which represent (1.6%).
2. 10 cases with factor VII deficiency which represent (2.6%).
3. 4 cases with factor XIII deficiency which represent (1.1%).
4. 6 cases with combined coagulation factor deficiency and platelete dysfunction which represent (1.6%).
Presenting symptoms of VWD cases: most common symptom bleeding tooth extraction (28.9%) each, followed by IM injection (12.8%), bleeding gum (11.8%), hematuria (3.7%), hematoma (3.7%) and menorrhgia (1.6%) all showing statistical significant difference.
Presenting symptoms of hemophilia A cases: most common symptom bleeding post traumatic (41.7%) each, followed by hematemsis (39.4%), bleeding gum (33.3%), menorrhgia (29.5%), melena (23.5%), and hematoma (22.7%) all showing statistical significant difference.
Presenting symptoms of hemophilia B cases: most common symptom bleeding gum (44.8%), each, followed by hematuria (41.1%), hematemsis (37.9%), post traumatic (34.5%), menorrhgia (31.1%) and purpura (27%) also all showing statistical significant difference.
In our study von willebrand disease represent the majority of cases, the number and percentage of cases with Von Willebrand’s disease was 187 which represent (50%) of whole cases of bleeding disorders.