Important note: Information in this article was accurate in 1990. The state of the art may have changed since the publication date.
PRIMARY IMMUNODEFICIENCY DISEASES
Fundamental Immunology. Second Edition. Paul WE, ed. New York, Raven, p. 1033-57, 1989.. Unique Identifier : AIDSLINE ICDB/90665508 Cooper MD; Butler JL; Div. of Developmental and Clinical Immunology, Univ. of Alabama,; Birmingham, AL 35294
Abstract:
Deficiencies of immune responsiveness can occur as either primary or secondary disorders. Secondary immunodeficiencies may be acquired as a consequence of treatment with immunosuppressive agents, nutritional deficiencies, and certain viral infections, the most notable of which is the HIV. Primary immunodeficiency diseases are inherited defects of the immune system that are typically manifested at birth or soon thereafter, although some become evident later in life. Frequent or unusually severe infections are the hallmark of immune system defects, the types of infections reflecting the nature of the defect. Autoimmune disorders and malignancies also occur more frequently in individuals with immunodeficiency diseases. Primary immunodeficiency diseases are reviewed, including severe combined immunodeficiency diseases (SCID: classical SCID, SCID with adenosine deaminase deficiency, SCID with purine nucleoside phosphorylase deficiency, SCID with B cells, SCID with major histocompatibility complex class II deficiency, reticular dysgenesia, and SCID in horses and mice), antibody deficiency diseases (congenital agammaglobulinemias, common variable immunodeficiency, immunoglobulin A [IgA] deficiency, selective deficiencies of other Ig isotypes, Ig deficiency with increased IgM, kappa-chain deficiency, transient hypogammaglobulinemia of infancy, thymoma and hypogammaglobulinemia, agammaglobulinemia following Epstein-Barr virus infection, and X-linked immunodeficiency in mice), and immunodeficiencies associated with other defects (Wiskott-Aldrich syndrome, ataxia-telangiectasia, third and fourth pharyngeal pouch syndrome, transcobalamin II deficiency, and nude athymic mice and rats). Because of their clinical importance, most of the known immunodeficiency diseases have been described in humans. Comparable disorders are rapidly being identified in other vertebrates, however, and murine immunodeficiency diseases are especially useful for pathogenetic studies. The defects in immunodeficiency diseases have provided valuable insight into normal development and function of the immune system. (376 Refs)
Keywords: Agammaglobulinemia/IMMUNOLOGY Animal *Chromosomes Human HIV Infections/IMMUNOLOGY Immunologic Deficiency Syndromes/GENETICS/*IMMUNOLOGY Mice Mice, Nude Rats Rats, Nude Sex Chromosome Abnormalities/IMMUNOLOGY MONOGRAPH REVIEW, ACADEMIC REVIEW 901030
M90A0680
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Important note: Information in this article was accurate in 1990. The state of the art may have changed since the publication date.
