Primary Immunodeficiency Disorders

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.

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 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.

Stiehm's Immune Deficiencies focuses on immunodeficiencies in children and adults. This book covers the many advances in the study of immunodeficiency. Stiehm's Immune Deficiencies includes 62 chapters covering topics such as newly described syndromes, genetic diagnosis, molecular abnormalities, newborn screening, and current therapies. Provides practical guidance to practitioners dealing with the day-to-day issues of diagnosis and management of immune deficient patients Covers both clinical management and scientific advances in one place Includes newly described disorders in various periodic updates to maintain the breadth of the reference

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.

This book provides a broad overview of the respiratory manifestations associated with Primary Immune deficiencies (PID) congenital genetic defects such as infections, chronic inflammation, autoimmunity, lymphoproliferation, allergic manifestations and rare forms of cancer. Since the most common site of involvement in PID is the lung, the pulmonologists (pediatrics or adult), Internists and General Practitioners may be among the first to recognize the pattern of pulmonary disorders, leading to diagnosis of PID. Pulmonary complications present a significant cause of morbidity and also mortality among patients suffering from different forms of PID. With the prevalence of lung infections and disease so high in PID patients, respiratory professionals will find this book to be an essential resource for diagnosing, managing and referring PID related pulmonary disorders in clinical practice.

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 Open Access edition of the European Society for Blood and Marrow Transplantation (EBMT) handbook addresses the latest developments and innovations in hematopoietic stem cell transplantation and cellular therapy. Consisting of 93 chapters, it has been written by 175 leading experts in the field. Discussing all types of stem cell and bone marrow transplantation, including haplo-identical stem cell and cord blood transplantation, it also covers the indications for transplantation, the management of early and late complications as well as the new and rapidly evolving field of cellular therapies. This book provides an unparalleled description of current practices to enhance readers’ knowledge and practice skills.

This book comprises a collection of categorized case-based questions, directed and meticulously selected to cover the most common and most important aspects of immunodeficiency diseases. Immunodeficiency disorders of infancy and childhood such as antibody deficiencies, phagocyte defects and defects in innate immunity are addressed among others. Each chapters starts with a brief of the initial presentation and lab data of the patient, followed by a series of 5-6 multiple choice questions (MCQs), leading the reader to the diagnosis and best of practice in a step-wise manner. This MCQ format along with precise, yet detailed answer ensures a quick, case-based, reality learning to the reader. This comprehensive MCQ series, is an essential reading material that a pediatric clinician, hematologist, immunologist, transplant specialist, or pulmonologist, can not afford to miss.

Immunobiology tells the story of the immune system. The book covers all of the material that comprises a typical immunology course. The Fifth Edition is an extensive revision which includes new material and major insights, improved logical progression of topics, and an emphasis on unifying principles. With clear, concise text and a full-color art program, this book continues to set the standard for a current and authoritative immunology textbook. Copyright © Libri GmbH. All rights reserved.

This book comprises a collection of case-based chapters, submitted by physicians and pediatricians in all specialties and meticulously refined and selected to cover the most common and important aspects of Pediatric Allergy knowledge. Commonly encountered disorders such as allergic rhinitis, food allergy, and atopic dermatitis are covered among many others. Each chapter starts with a brief of the initial presentation and lab data of the patient, followed by a series of 3-6 multiple choice questions (MCQs), leading the reader to the diagnosis and best of practice in a step-wise manner. Clinical pearls presented as "practical points", and the MCQ format along with detailed answers, makes Pediatrc Allergy an essential reading material that a pediatric allergologist cannot afford to miss.

This volume provides a comprehensive overview of critical care of the pediatric immunocompromised hematology-oncology patient. The text focuses on unique aspects of the pediatric immunocompromised patient that predisposes the child to significant illness, and presents critical care management strategies specific to the patient population. In addition to chapters on oncology, primary immune deficiency, immunocompromised hematology, and hematopoietic cell transplant patients, the book covers the changing landscape of ICU care, pharmacologic considerations, and psychological and social aspects of the critical care of hematology-oncology patients. Written by experts from a range of disciplines, Critical Care of the Pediatric Immunocompromised Hematology/Oncology Patient: An Evidence-Based Guide is a valuable resource for clinicians and practitioners who treat this patient population.

Autoimmunity is defined as an immune response against a self-antigen. This abnormal immune response can lead to tissue damage and to the development of autoimmune disease. From organ-specific autoimmune diseases, such as myasthenia gravis, to non-organ-specific, such as systemic lupus erythematosus, autoimmune diseases represent a heterogeneous group of disorders which affect approximately 6% of the population. The pathogenesis of many autoimmune diseases is complex and remains not completely understood. The aim of this book is to present current knowledge regarding pathogenic mechanisms of autoimmune diseases, clinical aspects of specific autoimmune diseases, like vitiligo, celiac disease and autoimmune liver disease, as well as insights regarding specific therapies.

Providing the practicing and trainee hematologist with a practical and immediately applicable compendium of answers the Clinical Manual of Blood and Bone Marrow Transplantation covers the spectrum of the hematopoietic cell transplant specialty, in particular practical issues in transplant patient care, and the set up and functioning of a transplant program. Supplies the practicing and trainee hematologist with a practical and immediately applicable compendium of answers to clinical questions Covers the spectrum of the hematopoietic cell transplant specialty, in particular practical issues in transplant patient care, and the set up and functioning of a transplant program Contains concise chapters written with a focus on tables, algorithms and figures to aid rapid referral Benefits from expert contributions from an international authorship

This book is a continuation of the efforts of InTech to expand the scientific know-how in the field of immunopathology and bring valuable updated information to medical professionals and researchers. It consists of chapters related to various approaches to investigate the unique role of the immune system in response to different clinical disorders. The international team of authors is the bonus of the book, reflecting the rapid development of immunology and new achievements in medical science. We firmly hope that the book will be an excellent manual and guideline for people dealing with biology, microbiology, immunology, virology, pharmacology, general and dental medicine, and health care, from students and postdocs to high-level specialists and university professors.

This book presents detailed state of the art knowledge on the humoral primary immunodeficiencies (PIDs), i.e., disorders arising from impaired antibody production due to defects intrinsic to B cells or defective interaction between B and T cells. There is extensive coverage of both basic science discoveries and the latest clinical advances in the field. The book is structured in accordance with the most recent classification of PIDs and also covers updates on the B cell immunological synapse. Readers will find comprehensive, in-depth descriptions of novel humoral PID genes and related clinical applications, mucosal B cells, and the various clinical phenotypes of humoral PIDs. Aspects such as differential diagnosis, clinical management in children and adults, and the role of vaccines are also addressed. The authors are all recognized experts from Europe, Australia, and the United States. Humoral Primary Immunodeficiencies will be of high value for immunologists, pediatricians, rheumatologists, oncologists, internists, and infectious disease specialists and will also be informative for MD and PhD students.

Diagnosing asthma requires knowledge of the multiple conditions that emulate it, and treatment requires addressing factors that modify the disease. This comprehensive, single reference will help practitioners identify and care for co-morbid conditions and improve asthma treatment.