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Chapter 9 - Developmental hemostasis II

from Section 2 - Special considerations in pediatric patients

Published online by Cambridge University Press:  18 December 2014

Marilyn J. Manco-Johnson
Affiliation:
University of Colorado
Neil A. Goldenberg
Affiliation:
The Johns Hopkins University School of Medicine
Marilyn J. Manco-Johnson
Affiliation:
Hemophilia and Thrombosis Center, University of Colorado, Denver
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Summary

Introduction

Early in the study of hemostasis it was discovered that the coagulation system of children, and especially of newborn and young infants, differed remarkably from that of adults in both quantitative and qualitative features, and yet these differences were not pathologic under normal physiologic conditions and represented an ontogeny of hemostasis [1–10]. Maureen Andrew, in particular, made substantial contributions to the field by systematically assessing various coagulation parameters from preterm infants to adolescents [11–14]. Studies in this field have predominantly reported functional assays of the coagulation proteins, presumably due to the fact that these assays are commonly used in clinical practice. Measurements of immunological levels of the coagulation proteins reported to date are fewer, but include: fibrinogen; prothrombin; von Willebrand factor; tissue plasminogen activator; antithrombin; protein C; free and total protein S, soluble thrombomodulin; soluble endothelial protein C receptor; ADAMTS13; and tissue factor pathway inhibitor [15–25]. Antigen levels are concordant with functional activity in the well newborn infant.

Development of many physiologic systems, including coagulation, is a continuous process over the age span. However, perhaps at no other life phase are dynamic changes in coagulation or manifestations of altered coagulation as dramatic as during the adaptation from fetal to neonatal life. Pediatric thrombosis is primarily a disorder of hospitalized children with underlying medical conditions and triggering provocations. Neonatal thrombosis is similarly a disorder of intensively supported newborn infants with various developmental, genetic and acquired conditions. Multiple components of the hemostatic system differ markedly between healthy newborn infants and healthy children and adults. However, healthy newborn infants rarely manifest excessive bleeding or clotting. In contrast, the sick newborn infant has a predilection to bleeding and clotting, with the rate of thrombosis the highest observed during childhood prior to puberty.

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Publisher: Cambridge University Press
Print publication year: 2015

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