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13 - Hemostatic abnormalities

Published online by Cambridge University Press:  10 August 2009

By
Manuela Albisetti ,
Maureen Andrew  and
Paul Monagle
Edited by
Pedro A. de Alarcón  and
Eric J. Werner
Foreword by
J. Lawrence Naiman
Pedro A. de Alarcón
Affiliation:
University of Tennessee
Eric J. Werner
Affiliation:
Eastern Virginia Medical School
J. Lawrence Naiman
Affiliation:
Stanford University School of Medicine, California
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Summary

Introduction

Hemostatic abnormalities can be considered as either congenital or acquired and can be classified as hemorrhagic or thromboembolic disorders. In newborns, the clinical presentation, diagnosis, and management of hemorrhagic and thromboembolic disorders differ from those in older infants and children, likely reflecting profound differences of the hemostatic system at birth. While severe congenital hemostatic defects usually present in the first hours to days of life with distinct symptoms in otherwise well newborns, acquired hemostatic disorders usually present in sick newborns with a variety of presentations and distinct etiologies that differ from older children and adults. In newborns, the diagnosis of hemostatic abnormalities based upon plasma concentrations of components of the hemostatic system requires age-appropriate reference ranges, because plasma concentrations of several procoagulant and inhibitor proteins are physiologically decreased at birth. The aim of this chapter is to discuss the clinical presentation, diagnosis, and management of the most common congenital and acquired hemostatic disorders in newborns.

Hemorrhagic disorders

Congenital hemorrhagic disorders

General information

Hemostatic proteins

For congenital deficiencies of components of the hemostatic system, both a severe and a milder form occur. Severe congenital deficiencies of prothrombin, factor V (FV), factor VII (FVII), factor VIII (FVIII), factor IX (FIX), factor X (FX), factor Ⅺ (FXI), fibrinogen, factor XIII (FXIII) and alpha2-antiplasmin (α2AP) all can present with bleeding in the first days of life [1–4]. Mild congenital deficiencies of these proteins usually do not cause bleeding at birth in otherwise healthy full-term newborns.

Type
Chapter
Information
Neonatal Hematology , pp. 310 - 348
Publisher: Cambridge University Press
Print publication year: 2005

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