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Chapter 5 - Immunodeficiency Diseases of the Neonate
- from Section II - Bone Marrow Failure and Immune 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 62-92
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Summary
Understanding the development of specific components of the neonatal immune system is critical to the understanding of the susceptibility of the neonate to specific pathogens [1]. With the increasing survival of extremely premature infants, neonatologists and other physicians caring for these newborns need to be aware of the vulnerability of this population. Furthermore, it is important for neonatologists to be able to differentiate between immune immaturity and the manifestations of a true primary immunodeficiency that present during the neonatal period. Failure to properly identify primary or acquired immunodeficiency diseases can result in delayed diagnosis and treatment, adversely affecting outcomes. This chapter will briefly define the immune immaturity of the neonate and a diagnostic approach for primary immune deficiency diseases that may present in the neonatal period.
15 - Immunodeficiency diseases of the neonate
- from Section V - Immunologic 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 255-276
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Summary
Significant progress has been made towards the understanding of the clinical implications of the immature neonatal immune system. With the increasing survival of extremely premature infants, neonatologists and other physicians caring for these patients must be aware of the vulnerability of this patient population. It is important for neonatologists to be able to differentiate between immune immaturity and a true primary immunodeficiency that may present during the neonatal period. Failure to properly identify primary immunodeficiency diseases can result in delayed diagnosis and treatment, which can significantly affect the outcome of the disease. This chapter will briefly define the immune immaturity of the neonate. More specifically, the immune deficiency syndromes that may present during the neonatal period are discussed followed by a stepwise approach on how to properly diagnose and manage neonates with suspected immunodeficiency diseases.
Immaturity of the neonatal immune system
The immaturity of a neonate’s immune response places them at an increased risk for serious infection. An understanding of the development of the neonatal immune system is essential in order to be able to differentiate the clinical manifestations of infection associated with immaturity from those that identify a specific acquired or primary immunodeficiency disease. For further detail on the development of the immune system, the reader should refer to Chapter 3. The immune defects associated with immaturity and their associated susceptibility to specific types of infections are summarized in Table 15.1.
Management of children undergoing cardiac transplantation with high Panel Reactive Antibodies
- Alfred Asante-Korang, Jeffrey P. Jacobs, Jeremy Ringewald, Jennifer Carapellucci, Kristin Rosenberg, Daniel McKenna, Jorge McCormack, Ivan Wilmot, Abigail Gjeldum, Mayra Lopez-Cepero, John Sleasman
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- Journal:
- Cardiology in the Young / Volume 21 / Issue S2 / 13 December 2011
- Published online by Cambridge University Press:
- 13 December 2011, pp. 124-132
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Highly sensitised children in need of cardiac transplantation have overall poor outcomes because of increased risk for dysfunction of the cardiac allograft, acute cellular and antibody-mediated rejection, and vasculopathy of the cardiac allograft. Cardiopulmonary bypass and the frequent use of blood products in the operating room and cardiac intensive care unit, as well as the frequent use of homografts, have predisposed potential recipients of transplants to allosensitisation. The expansion in the use of ventricular assist devices and extracorporeal membrane oxygenation has also contributed to increasing rates of allosensitisation in candidates for cardiac transplantation. Antibodies to Human Leukocyte Antigen can be detected before transplantation using several different techniques, the most common being the “complement-dependent lymphocytotoxicity assays”. “Solid-phase assays”, particularly the “Luminex® single antigen bead method”, offer improved specificity and more detailed information regarding specificities of antibodies, leading to improved matching of donors with recipients. Allosensitisation prolongs the time on the waiting list for potential recipients of transplantation and increases the risk of complications and death after transplantation. Aggressive reduction of antibodies to Human Leukocyte Antigen in these high-risk patients is therefore of vital importance for long-term survival of the patient and cardiac allograft. Strategies to decrease Panel Reactive Antibody or percent reactive antibody before transplantation include plasmapheresis, intravenous administration of immunoglobulin, and specific treatment to reduce B-cells, particularly Rituximab. These strategies have resulted in varying degrees of success. Antibody-mediated rejection and cardiac allograft vasculopathy are two of the most important complications of transplantation in patients with high Panel Reactive Antibody. The treatment of antibody-mediated rejection in recipients of cardiac transplants is largely empirical and includes the use of high-dose corticosteroids, plasmapheresis, intravenous administration of immunoglobulins, anti-thymocyte globulin, and Rituximab. Cardiac allograft vasculopathy is believed to be secondary to chronic complement-mediated endothelial injury and chronic vascular rejection. The use of proliferation signal inhibitors, such as sirolimus and everolimus, has been shown to delay the progression of cardiac allograft vasculopathy. In some non-sensitised recipients of cardiac transplants, the de novo formation of antibodies to Human Leukocyte Antigen after transplantation may increase the likelihood of adverse clinical outcomes. The use of serial testing for donor-specific antibodies after cardiac transplantation may be advisable in patients with frequent episodes of rejection and patients with history of sensitisation. Allosensitisation before transplantation can negatively influence outcomes after transplantation. A high incidence of antibody-mediated rejection and graft vasculopathy can result in graft failure and decreased survival. Current strategies to decrease allosensitisation have helped to expand the pool of donors, improve times on the waiting list, and decrease mortality. Centres of transplantation offering desensitisation are currently using plasmapheresis to remove circulating antibodies; intravenous immunoglobulin to inactivate antibodies; cyclophosphamide to suppress B-cell proliferation; and Rituximab to deplete B-lymphocytes. Similar approaches are also used to treat antibody-mediated rejection after transplantation with promising results.
12 - Immunodeficiency diseases of the neonate
- 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 280-309
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Summary
Introduction
Significant progress has been made towards the understanding of the clinical implications of the neonatal immune system and its immaturity. With the increasing survival of extremely premature infants, neonatologists and other physicians caring for these patients must be aware of the vulnerability of this select patient population. It is also important for neonatologists and pediatricians to be able to differentiate between immune immaturity and a true primary immunodeficiency that may present during the neonatal period. Failure to identify properly primary immunodeficiency diseases can result in delayed diagnosis and treatment, which can significantly affect the outcome of the disease. This chapter defines the immune immaturity of the neonate and how it impacts susceptibility to neonatal infection. It will describe the specific immune deficiency syndromes that may present during the neonatal period. Finally, the diagnosis and management of neonatal immunodeficiency diseases will be discussed in length in order to provide the reader with the proper approach and management guidelines to care adequately for these individuals.
Immaturity of the neonatal immune system
The immaturity of a neonate's immune response places the neonate at an increased risk for serious infection. An understanding of the development of the neonatal immune system is essential in order to be able to differentiate the clinical manifestations of infection associated with immaturity from those that identify a specific acquired or primary immunodeficiency disease. The focus of this section is to define how the immune immaturity of the neonate impacts susceptibility to infection.