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The number of diagnosed cases of primary immunodeficiency diseases (PIDs) – a group of inborn disorders of the immune system – is growing rapidly, but misdiagnosis or late diagnosis still occurs in a significant number of patients, with serious consequences. This is the second edition of a practical reference textbook on PIDs that has been widely welcomed by scientists and clinicians from around the world. The new edition has been extensively revised to reflect advances in knowledge and includes various PIDs not previously covered. For each disease, information is provided on definition, etiology, clinical manifestations, diagnosis, and management. This book will represent an ideal resource for specialists when engaging in diagnosis, clinical decision-making, and treatment planning. It will also prove invaluable for doctors in training and other physicians and nurses who wish to learn more about PIDs.
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.
The second edition of Primary Immunodeficiency Diseases presents discussions of gene identification, mutation detection, and clinical and research applications for over 100 genetic immune disorders--disorders featuring an increased susceptibility to infections and, in certain conditions, an icreased rate of malignancies and autoimmune disorders. Since the publication of the first edition, a flurry of new disease entities has been defined and new treatment regimens have been introduced, the most spectacular being successful treatment by gene therapy for two genotypes of combined immunodeficiency. The first edition marked a historic turning point in the field of immunodeficiencies, demonstrating that many of the disorders of the immune systam could be understood at a molecular level. This new edition can proudly document the tremendous pace of progress in dissecting the complex immunologic networks responsible for protecting individuals from these disorders.
Primary immunodeficiency diseases (PIDs) are a heterogeneous group of inherited disorders characterized by different defects in the development and function of the immune system. This book aims to increase the clinical awareness and knowledge of practicing clinicians regarding the diagnosis and management of PIDs. In order to achieve this goal, about 90 cases drawn from real life are presented, along with approximately 300 related questions. The selected case reports are the result of the invaluable cooperation of more than 40 scientists in the field of immunodeficiency. They focus both on the presenting features of patients with PIDs and on the required further investigation and management. Each of the numbered cases is followed by the questions, their answers, and additional discussion. Each question focuses on a particular aspect of the PID under consideration, and the topics covered include clinical diagnosis, laboratory findings, molecular mechanisms, and therapy.
Awareness among clinicians about PIDs, which consist of more than 400 different entities, plays an important role in ensuring that patients receive a timely diagnosis. Furthermore, clinicians who are educated about PIDs can give their patients access to optimal management of their condition, thus helping the patient achieve a better quality-of-life and long-term prognosis. Inborn Errors of Immunity: A Practical Guide provides the most up-to-date information for busy students, nurses, clinical residents, practicing physicians, and even basic researchers. Readers will benefit from a well-structured breakdown of complicated PID diseases, including approaches to their clinical signs/symptoms and immunologic/laboratory findings. - Presents valuable contribution of more than 40 expert chapter authors, from top centers spanning five continents, each in a specific PID field - Covers various aspects of PID using updated clinical guidelines and standard stepwise pipelines - Focuses on the latest developments in the molecular diagnosis and pathogenesis of diseases, with easy explanation and schematic representation of defective signaling pathways - Includes dedicated sections for clinical features and immunological tests with carefully-curated figures of PID manifestations, imaging, and histological/pathological illustrations to create the first PID medial-color atlas - Summarizes the updated conventional and specific treatments and follow-up notes for different PID diseases
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.
26 real-life cases illustrate the applications of basic immunology in clinical settings May be utilized alone or as a companion to Immunology: A Short Course, 7th Edition by Richard Coico and Geofftry Sunshine (ISBN 9781118396919) Each case study is introduced by clearly written descriptions of the major immunological disorders Full colour photographs and illustrations complement complete presentation of real data Includes complete set of problems and discussion questions for each chapter
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 therapiesHighlights the importance of studying humans to understand mechanisms of disease that affect humans
A full-color, case-based review of the essentials of pathophysiology--covering all major organs and systems The goal of this trusted text is to introduce you to clinical medicine by reviewing the pathophysiologic basis of 120 diseases (and associated signs and symptoms) commonly encountered in medical practice. The authors, all experts in their respective fields, have provided a concise review of relevant normal structure and function of each body system, followed by a description of the pathophysiologic mechanisms that underlie several common diseases related to that system. Each chapter of Pathophysiology of Disease concludes with a collection of case studies and questions designed to test your understanding of the pathophysiology of each clinical entity discussed. These case studies allow you to apply your knowledge to specific clinical situations. Detailed answers to each case study question are provided at the end of the book. This unique interweaving of physiological and pathological concepts will put you on the path toward thinking about signs and symptoms in terms of their pathophysiologic basis, giving you an understanding of the "why" behind illness and treatment. Features 120 case studies (9 new) provide an opportunity for you to test your understanding of the pathophysiology of each clinical entity discussed Checkpoint questions provide review and appear in every chapter Updates and revisions throughout this new edition reflect the latest research and developments Numerous tables and diagrams encapsulate important information Updated references for each chapter topic Pathophysiology of Disease is a true must-have resource for medical students preparing for the USMLE Step 1 exam, as well as students engaged in their clerkship studies. House officers, nurses, nurse practitioners, physicians’ assistants, and allied health practitioners will find its concise presentation and broad scope a great help in facilitating their understanding of common disease entities.