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Chapter 18 - Bleeding Disorders
- from Section VI - Hemostatic Disorders
- Edited by Pedro A. de Alarcón, Eric J. Werner, Robert D. Christensen, University of Utah, Martha C. Sola-Visner, Harvard University, Massachusetts
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- Book:
- Neonatal Hematology
- Published online:
- 30 January 2021
- Print publication:
- 18 February 2021, pp 293-311
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Summary
Bleeding symptoms presenting in the neonatal period usually present a diagnostic and therapeutic challenge for treating physicians. Bleeding disorders may be due to either congenital or acquired coagulation disorders, and may be related to mortality or long term morbidity when not appropriately and timely diagnosed. While severe congenital coagulation defects usually present in the first hours to days of life with distinct symptoms in otherwise well newborns, acquired coagulation 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 coagulation 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 clinical presentation, diagnosis, and management of the most common congenital and acquired bleeding disorders in newborns, excluding platelet disorders.
17 - Bleeding disorders
- from Section VI - Hemostatic disorders
- Edited by Pedro de Alarcón, Eric Werner, Robert D. Christensen
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- Book:
- Neonatal Hematology
- Published online:
- 05 February 2013
- Print publication:
- 10 January 2013, pp 286-302
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Summary
Introduction
Bleeding symptoms in the neonatal period usually present a diagnostic and therapeutic challenge for treating physicians. Bleeding disorders may be due to either congenital or acquired coagulation disorders, and may be related to mortality or long-term morbidity when not appropriately and timely diagnosed. While severe congenital coagulation defects usually present in the first hours to days of life with distinct symptoms in otherwise well newborns, acquired coagulation 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 coagulation 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 clinical presentation, diagnosis, and management of the most common congenital and acquired bleeding disorders in newborns, excluding platelet disorders.
General information
Developmental hemostasis
Components of the hemostatic system are already synthesized by the fetus starting at 10 weeks’ gestational age. At birth, all factors of the coagulation and fibrinolytic system are present and measurable. However, the concentration of several factors differs significantly from older children and adults. In the coagulation system, plasma concentrations of the vitamin K-dependent factors (F), contact factors, and the capacity to generate thrombin are decreased in newborns as compared to adults, while other factors such as fibrinogen, FV, FVIII, and FXIII are similar or increased at birth (1–3). Plasma concentrations of the inhibitors antithrombin, heparin cofactor II, protein C, and protein S are decreased at birth up to 50% of older children and adult values. By contrast, the plasma concentration of α2-macroglobulin in newborns is increased approximately twice compared with adult values.
13 - Hemostatic abnormalities
- Edited by Pedro A. de Alarcón, University of Tennessee, Eric J. Werner
- Foreword by J. Lawrence Naiman, Stanford University School of Medicine, California
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- Book:
- Neonatal Hematology
- Published online:
- 10 August 2009
- Print publication:
- 18 August 2005, pp 310-348
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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.