Book contents
- Frontmatter
- Contents
- Contributing Authors
- Preface to the Third Edition
- Preface to the First Edition
- SECTION I PATHOPHYSIOLOGY OF PEDIATRIC LIVER DISEASE
- SECTION II CHOLESTATIC LIVER DISEASES
- SECTION III HEPATITIS AND IMMUNE DISORDERS
- 17 Acute and Chronic Viral Hepatitis
- 18 Autoimmune Hepatitis
- 19 Sclerosing Cholangitis
- 20 Drug-Induced Liver Disease
- 21 Liver Disease in Immunodeficiencies
- SECTION IV METABOLIC LIVER DISEASE
- SECTION V OTHER CONDITIONS AND ISSUES IN PEDIATRIC HEPATOLOGY
- Index
- Plate section
- References
21 - Liver Disease in Immunodeficiencies
from SECTION III - HEPATITIS AND IMMUNE DISORDERS
Published online by Cambridge University Press: 18 December 2009
- Frontmatter
- Contents
- Contributing Authors
- Preface to the Third Edition
- Preface to the First Edition
- SECTION I PATHOPHYSIOLOGY OF PEDIATRIC LIVER DISEASE
- SECTION II CHOLESTATIC LIVER DISEASES
- SECTION III HEPATITIS AND IMMUNE DISORDERS
- 17 Acute and Chronic Viral Hepatitis
- 18 Autoimmune Hepatitis
- 19 Sclerosing Cholangitis
- 20 Drug-Induced Liver Disease
- 21 Liver Disease in Immunodeficiencies
- SECTION IV METABOLIC LIVER DISEASE
- SECTION V OTHER CONDITIONS AND ISSUES IN PEDIATRIC HEPATOLOGY
- Index
- Plate section
- References
Summary
Man and microbes are committed to a perennial evolutionary conflict in which the human immune system represents a powerful tool of protection against invasion. The liver plays an important role in the immune defense because of its central position adjacent to the gastrointestinal tract, representing the first line of defense against ingested pathogens and various antigens from food.
There are two types of immune response innate and adaptive. Innate immunity represents the first line of immune defense in which cells such as phagocytes, natural killer (NK) cells, and NK T cells recognize highly conserved antigens from the invading microorganisms and provide a prompt nonspecific inflammatory response. The principal intrahepatic defenders are Kupffer cells – resident macrophages, strongly supported by the action of NK cells, previously also known as Pit cells, which represent approximately 50% of the lymphocyte pool in a healthy liver. In contrast, the adaptive immune system is more phylogenetically advanced and includes more highly specialized cells such as T and B lymphocytes, produced and differentiated in the lymphoid organs. On stimulation, these cells undergo sophisticated processes of immune diversification enabling them to mount specific immune responses to different invading antigens from the environment. These events are much slower and involve mechanisms such as cell-to-cell interaction, proliferation of B cells, production of antibodies and cytokines, and activation of effector cytotoxic cells.
- Type
- Chapter
- Information
- Liver Disease in Children , pp. 513 - 528Publisher: Cambridge University PressPrint publication year: 2007
References
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