Developmental hemostasis: relevance to hemostatic problems during childhood.

Congenital and acquired hemostatic disorders present during childhood. An accurate diagnosis is critically important in order to implement optimal therapy. Over the last century, several investigators have measured plasma concentrations of hemostatic components in newborns and compared the results to adult values. Clinically significant differences exist for many hemostatic components. Recently, three large studies in more than 400 healthy children have provided reference ranges for hemostatic components throughout childhood. Together, these studies provide insight into the regulation of coagulation and fibrinolysis in children in physiologic and pathologic states. Some examples of the influence of age on hemostasis are: (1) the diagnosis of some congenital factor deficiencies, based on plasma levels, can be difficult due to physiologically low values; (2) despite very low levels of many inhibitors of hemostasis, thrombotic complications are rare; (3) the interaction of anti-coagulants and thrombolytic agents is profoundly influenced by the relative immaturity of hemostasis at birth; and (4) in contrast to the risk of thrombosis, healthy infants are at risk for vitamin K deficiency bleeding due to poor transport of vitamin K across the placenta and plasma concentrations of the vitamin K dependent proteins of less than 50% of adults values. The following review discusses the age dependency of hemostasis during childhood and the effect of the physiologically immature system on the diagnosis and treatment of hemostatic disorders.