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Immunological disorders in infants & children, 5th edition
  1. A Jones

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    Edited by E Richard Stiehm, Hans D Ochs, Jerry A Winkelstein. Philadelphia: Elsevier Saunders, 2004, pp 1458, £174.00 (hardcover). ISBN 0 7216 8964 7


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    The latest edition of this definitive text is certainly not intended for cover-to-cover reading, but aims to contain “everything you always wanted to know (and probably much more) about paediatric immunological disorders”. It contains a vast amount of information written by many of the leading names in the field, and undoubtedly succeeds in its aim as far as any textbook covering a rapidly evolving field can hope to do.

    The first edition was published in 1973, and this fifth edition has been published just one year after the death of Robert Good, author of the first chapter of the first edition, “Crucial experiments of nature that have guided analysis of the immunologic apparatus”. These “experiments of nature”, as well as a great deal of basic science, have since then continued to provide insights into the immense complexity of the immune system. The advances in understanding of both basic and clinical immunology even since publication of the last edition in 1996 have been extensive, and this is reflected in the addition of much new material. The book is, as previously, intended for a wide readership, including paediatric sub-specialists, general paediatricians, paediatric trainees, and medical students. It will also be of considerable interest to adult immunologists. It is divided into four sections: Development and Function of the Immune System, Primary Immunodeficiencies, Secondary Immunodeficiencies, and Immunologic Aspects of Paediatric Illness.

    The section on ontogeny and fundamental immunology may appear relevant only to sub-specialists. However, increasing recognition of the role of various forms of immunological dysregulation in the pathogenesis of a wide variety of disorders in many paediatric disciplines means that these chapters are a valuable reference resource. New chapters in the first section include one on innate immunity, which contains much recent information about mechanisms of natural immunity, such as the mannose binding lectin system, Toll-like receptors, defensins, and the roles of the major cytokines. Also new is a chapter on the immunology of pregnancy.

    Detailed discussions of over 100 defined primary immunodeficiencies in Section II are preceded by a useful overview, including discussion of clinical immunology investigations. The huge expansion in understanding of the basic mechanisms underlying these disorders is reflected in the increase from one to four chapters on combined and isolated T cell deficiencies. Many recently identified molecular defects are discussed. Likewise the chapter on primary antibody deficiency includes descriptions of more molecularly defined disorders, including the four defined forms of hyper-IgM syndrome. The X linked form (CD40 ligand deficiency) arguably belongs with T cell disorders given that many of its manifestations reflect the basic T cell defect rather than antibody deficiency, but this is a minor point. A completely new chapter for this edition focuses on disorders of apoptosis, which manifest as autoimmune lymphoproliferative syndrome.

    The third and fourth sections of the book are likely to be of most general interest. An extensive section on the immunodeficiency of immaturity may be of particular interest to neonatologists. Discussions of every aspect of neonatal immunology are each followed by a succinct summary for those without the time or the inclination to struggle with basic immunology. A wide variety of genetic syndromes with immunodeficiency as a component are described in another new chapter, and there are expanded discussions of immunodeficiency associated with malnutrition, splenic deficiency, and paediatric HIV infection, as well as immunological aspects of surgical and anaesthetic stress. The final section covers immunological aspects of many paediatric disorders affecting virtually every system, including descriptions of the periodic fever syndromes, the molecular basis for several of which have recently been elucidated, infection in immunocompromised children, immunisation, and comprehensive coverage of both solid organ and bone marrow transplantation in children.

    This is an impressive reference text that provides an appealing balance between specialised descriptions of complex immunology and rare primary immunodeficiencies, and the roles played by immune mechanisms in a wide variety of both rare and relatively frequent paediatric disorders. Although it is inevitably already out of date—for example, in the areas of most recent antifungals and monoclonal antibody therapies—this does not detract from its value. It will undoubtedly retain the position of the major comprehensive reference work in paediatric immunology.

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