الفهرس 
Coagulation DisordersOnly 14 pages are availabe for public view
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Abstract
Background: Rare bleeding disorders are autosomal recessive disorders. Their frequency in the general population ranges from 1:500.000 to 1:2 millions. In countries with a high rate of consanguineous marriages RBDs occur more frequently. Patients affected by RBDs have a wide spectrum of clinical symptoms that vary from a mild or moderate bleeding tendency to potentially serious or life-threatening hemorrhages. Current treatment is based on both replacement therapy and non-transfusional treatment.
Objectives: The present study aimed to assess the prevalence of various bleeding disorders in Children’s Hospital, Ain Shams University.
Methods: This study was carried in a retrospective manner (hospital based registry) to assess the types of bleeding disorders along a period of 14 years starting ( 1994 to 2007) for the patients who were recorded in the hematology clinic, Children’s Hospital, Ain Shams University.
Results: A total of 687 cases of the 2949 cases attended pediatrics’ hematology clinic in 14 years were characterized to have bleeding disorders. Inherited coagulation disorders were observed in 187 (27.2%) of the cases. Hemophilia A 132 (70.6%) of the cases, hemophilia B 26 (13.9%) of cases, and factor I deficiency in 4 (2.1%) patients, factor V deficiency in 3 (1.6%), factor X deficiency in 8 (4.2%), factor VII deficiency in 5 (2.6%), factor XIII deficiency was seen in 2 (1.1%) and combined factor deficiency 4 (2.1%) of patients and unclassified coagulation disorders 3 cases. Inherited platelet dysfunction was seen in 96 (13.9%) of the patients. Von Willebrands disease in 33(6.6%) patients, Glanzman’s thrombasthenia in 56 (11.2%) patients, Bernard Soulier Syndrome in 5(1%) patients and Chediak Higashi Syndrome in 2(0.4%) patients. In 30 (6%) patients, the platelet function defect could not be classified into any specific subtypes. Median age in coagulation disorders was 33 months; while in platelet disorders was 72 months. Cases with platelets disorders had higher rate of female cases than those with coagulation disorders.
Conclusion: The development of registries will enable the different manifestations of theses diseases to be explored