الفهرس 
Arabic Summary
Primary haemostasisOnly 14 pages are availabe for public view
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Abstract
Inherited bleeding and thrombotic disorders are contraposed facets of genetically transmitted disease affecting the basic physiological mechanisms of haemostasis. Haemostasis involves several interactive and interdependent biological pathways including reciprocally acting mechanisms between the vasculature and its supporting matrix. In two distinct intravascular mechanisms, primary and secondary haemostasis, that produce a haemostatic plug of platelets and fibrin that arrests bleeding.
Coagulation factor inhibitors and components of the fibrinolytic system regulate these processes and prevent the dissemination of clot formation to sites distant from vessel injury. The hereditary bleeding disorders are due to genetic abnormalities affecting one or several components operating in primary or secondary haemostasis leading to impaired haemostatic plug formation. In contrast, the hereditary thrombotic disorders (thrombophilia) are the result of genetic abnormalities acting inhibitors and fibrinolysis promoting uncontrolled clot generation and thrombus formation. An integrated approach to the molecular genetics, pathophysiology and essential clinical and diagnostic features of these disorders, as well as their comprehensive management, should ensure favourable and cost-effective outcomes .
Although some bleeding is the natural consequence of any invasive procedure, the assessment should consist of a detailed history and physical examination, screening tests and specific assays. In the absence of a definitive diagnosis from this assessment, the process raises awareness as to the possibility that haemostatic disturbances could occur and require treatment. The assessment aims to provide a measure of possible clinical severity of previously known or newly detected disorders.
Though accurate diagnosis of different types ,frequency and suspected danger we can determine best treatment among available different means of treatment such as medical treatment which classified into several groups contains wide range of drugs suitable specifically for different types of congenital neonatal hemorrhages .
General principles for the management of the inherited bleeding disorders related to phenotype and include:
1. Replacement regimens using cellular components or exogenous purified and treated plasma derived or recombinant proteins.
2. The use of pharmacological agents that increase the production of endogenous proteins or cellular components.
3. The administration of agents that bypass abnormalities in the coagulation cascade or promote platelet aggregation through thrombin generation.
Finally efforts continue in the direction of achievement better diagnosis and control of neonatal hemorrhages with less side effects and reducing its danger