for a given clinical situation are limited in children due to the rarity of this diagnosis An individualized treatment plan for patients with additional thrombotic risk factors is appropriate DISORDERS OF HEMOSTASIS Goals of Treatment Achieving hemostasis in the setting of spontaneous or traumatic bleeding is paramount Additionally, taking appropriate preemptive steps prior to procedural intervention for patients with ongoing or potential hemorrhage is critical CLINICAL PEARLS AND PITFALLS Factor replacement is the primary goal in any hemophilia patient with suspected or known bleeding Measured levels of factor VIII and factor IX typically predict clinical severity; however, levels for other factor deficiencies including VII and XI not reliably correlate with bleeding phenotype Current Evidence The most common inherited bleeding disorders are VWD, factor VIII deficiency (hemophilia A), and factor IX deficiency (hemophilia B) These bleeding disorders are reviewed in more detail in the following sections Other rare inherited disorders include isolated defects or deficiencies in specific factor proteins which contribute to the initiation of clot formation such as factor II, factor V, factor VII, factor X, factor XI, proteins responsible for clot stabilization such as factor XIII, and proteins which counter the fibrinolytic pathway such as plasminogen activator inhibitor type-1 (PAI-1) Each of these deficiencies has a clinically variable phenotype This combined with their rarity has hindered the development of clinical practice guidelines for rare bleeding disorders Once a diagnosis is made, therapeutic decisions must be tailored to the individual patient based on bleeding severity and location Acquired defects of hemostasis are uncommon in the pediatric population, but consider these disorders in the setting of acute onset of an unexpected bleeding episode in the absence of any personal or family history of bleeding Among the more common acquired causes are DIC, which can arise in the setting of sepsis or severe trauma, uremia and drug-induced platelet dysfunction, and decreased factor synthesis due to liver disease and cholestasis Clinical Considerations Clinical Recognition Severe congenital disorders of hemostasis typically present during infancy Mild and moderate disorders may go undetected until later childhood or even adulthood when