Immunologic Deficiency Syndrome

Topics include: "Genetic Diagnosis ofPrimary Immunodeficiencies","IPEX syndrome and Treg deficiency in primaryimmunodeficiency","DiGeorge syndrome and other thymic hypoplasias","Common VariableImmunodeficiency", "Hyper IgE syndromes","Hyper IgM syndromes","Hematophagocyticlymphohistiocytoses", "Defects of the Toll LikeReceptor System", "Congenital Neutropenias", "Advances in Severe Combined Immunodeficiency", "Genetic Defects of Apoptosis and PrimaryImmunodeficiency", "Principles of and Advances inimmunoglobuin replacement therapy for primary immunodeficiency", "Gene therapy for primary immunodeficiency"

This third edition of Primary Immunodeficiency Diseases provides readers with the historic and scientific background, clinical presentations, immunologic characteristics, and the molecular/genetic underpinnings of this rapidly enlarging class of diseases.

This book provides a broad clinical overview of primary and secondary immunodeficiencies nested in clinical cases that will help the reader understand the approach to evaluation, diagnosis, and management of these challenging cases. Chapters begin with a comprehensive overview of immunodeficiencies to ground the reader in practical knowledge of the field and these complex conditions. Chapters cover B lymphocyte immunodeficiency, T cell immunodeficiency, immune dysregulation syndromes, and innate immune defects. They discuss a range of treatment options including gene therapy or bone marrow transplant. The book continues with a discussion on secondary immunodeficiencies and their treatment. Each chapter was written by authors with expertise related to different immunodeficiency disorders and provide a succinct overview of pathomechanisms, diagnosis, and treatment of a specific condition. Primary and Secondary Immunodeficiency will be an excellent resource for practicing allergists, hematologists, clinical immunologists, fellows, residents, and other clinicians who work with immunodeficiency patients.

Primary Immunodeficiency Disorders: A Historic and Scientific Perspective provides a complete historical context that is crucial for students and researchers concerned with primary immunodeficiency. When researchers have a poor understanding of the way we arrived where we are in research, they can miss important points about a disease, or miss out on how to approach new diseases. This historical knowledge of research can assist greatly by showing how it was done in the past, demonstrating the successes and failures, so that it can be done better in the future. This book provides an understanding of the process going from clinical problem to lab and back to the clinic, based on historical experiences. Its chapters proceed from the discovery of the T and B cell lineages through the first BMT for immunodeficiency disorder; lab investigation and gene therapy for PID; the discovery of the gene for AT and its function; understanding cytokine defects; and many other stops along the way. Facilitates communication among physicians and other investigators concerned with immunological and inflammatory diseases Summarizes for the first time all the known facts from 60 years of primary immunodeficiency research, and teaches how an important field in medicine was established Provides stimulating discussions on developing new medical therapies Highlights the importance of studying humans to understand mechanisms of disease that affect humans

The AIDS epidemic has popularized immune deficiency and has led to a rapid increase in the funding for research into the effect of viruses on immunity. There is now a real possibility of finding out whether some of the rare, so-called 'primary' immunodeficiency syndromes of children and adults, first described in the 1950s, may have a viral aetiology. Most of these syndromes have already been extensively reviewed more than once over the past 10 years, and their diagnosis and management is now included in most standard medical textbooks. In this volume, I have chosen to highlight what I consider to be the potentially exciting aspects of immunodeficiency in humans, hopefully providing clinicians and medical academics with some insight into the recent breakthroughs and disappointments. There are three chapters discussing viral interactions with the immune system. Although much has been written about AIDS already, I make no apology for including an extensive review of both the clinical and laboratory aspects of this disease. AIDS is an important precedent, and it is likely that other less pathogenic retroviruses will eventually turn dut to cause some of the rarer immunodeficiency disorders in children and adults. For instance, it has long been suspected that viruses may cause some of the non-familial types of severe combined immunodeficiency in infants; in fact HIV-I infection now has to be considered in the differential diagnosis.

This book presents case histories to illustrate in a clinical context essential points about the mechanisms of immunity. It includes cases that illustrate both recently discovered genetic immunodeficiencies and some more familiar and common diseases with interesting immunology.

Stiehm’s Immune Deficiencies: Inborn Errors in Immunity, Second Edition, is ideal for physicians and other caregivers who specialize in immunology, allergies, infectious diseases and pulmonary medicine. It provides a validated source of information for care delivery to patients, covering approaches to diagnosis that use both new genetic information and emphasize screening strategies. Management has changed dramatically over the past five years, so approaches to infection and autoimmunity are emphasized in an effort to improve outcomes and disseminate new information on the uses of targeted therapy. Covers immune deficiencies that are presented in a practical way, providing helpful information for active clinicians Fills an increasingly deep gap in the information available to clinicians Presents both clinical management and scientific advances for immune deficiencies Provides a primary resource for physicians in the field of immunodeficiencies Includes website access to a range of videos relevant to the topics discussed