Pediatric clinics of North America
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Pediatr. Clin. North Am. · Dec 2013
ReviewInherited abnormalities of coagulation: hemophilia, von Willebrand disease, and beyond.
Bleeding disorders are broadly classified into primary and secondary hemostatic defects. Primary hemostatic disorders (disorders of platelets and von Willebrand factor) mainly result in mucocutaneous bleeding symptoms such as epistaxis, menorrhagia, petechiae, easy bruising, and bleeding after dental and surgical interventions. Secondary hemostatic disorders (congenital or acquired deficiencies of coagulation factors) typically manifest with delayed, deep bleeding into muscles and joints. This article provides a generalized overview of the pathophysiology, clinical manifestations, laboratory abnormalities, and molecular basis of inherited abnormalities of coagulation with a focus on hemophilia, von Willebrand disease, and rare inherited coagulation disorders.
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Pediatr. Clin. North Am. · Dec 2013
ReviewDevelopmental hemostasis: clinical implications from the fetus to the adolescent.
The coagulation system involves a dynamic group of procoagulation and anticoagulation proteins that appear early in fetal life and whose levels change throughout childhood and into the teenage years. This process is called developmental hemostasis. Developmental hemostasis creates unique challenges for clinicians affecting the diagnosis and treatment of coagulation disorders during early childhood. The objective of this review is to assist pediatricians in understanding the coagulation system in fetal life and childhood and to provide guidance for interpreting basic coagulation testing, which will result in an improved ability to diagnose and treat patients with hemostatic and thrombotic disorders.