Pediatric Coagulation Disorders.

Abstract

1. Vilmarie Rodriguez, MD* 2. Deepti Warad, MBBS* 1. *Division of Pediatric Hematology-Oncology, Mayo Clinic Children’s Center, Mayo Clinic Comprehensive Hemophilia Center, Mayo Clinic, Rochester, MN. Clinicians are often challenged in primary practice with patients who present with potential hemostatic disorders. Determining which laboratory tests to order and when to refer a patient to a pediatric hematologist is of vital importance. After completing this article, the reader should be able to: 1. Describe the physiology of hemostasis in the pediatric patient. 2. List clinical signs and symptoms suggestive of a congenital or acquired bleeding disorder. 3. Understand laboratory testing and indications in the diagnosis of a bleeding disorder and timing of subspecialty referral. 4. Describe the clinical management of bleeding disorders. 5. Become familiar with congenital and acquired thrombophilic disorders and their diagnosis and management recommendations. Hemostasis in the pediatric patient evolves as the child grows and matures, and normal adult laboratory values are not often the norm for a child. This article provides a summary of the pathophysiology of hemostasis (eg, bleeding and thrombosis disorders) and reviews the basic principles of history and physical findings to provide the primary care practitioner with the necessary tools for initial evaluation of a pediatric patient with a suspected coagulation disorder. A basic understanding of when to test a patient for a coagulation disorder or make a proper referral to a pediatric hematologist will result in optimal care. Hemostasis is a complex process that requires a balance between maintaining blood in a fluid state and addressing areas of tissue injury in which a local response is generated at the site of vascular endothelial injury to promote healing and prevent hemorrhage. This process requires the interaction of the vascular endothelium, platelets, and coagulation factors. Diminished or dysfunctional activity of any of the components of the …