Inborn Errors of Immunity

"This book provides a broad, comprehensive overview of primary immunodeficiencies (PIDs) and is a useful resource for clinical immunologists and other medical providers. The book is divided into 10 sections with the first 9 sections dedicated to the main classifications of PIDs—combined immunodeficiencies, combined immunodeficiencies with syndromic features, predominantly antibody deficiencies, diseases of immune dysregulation, congenital defects of phagocytes, defects of intrinsic and innate immunity, autoinflammatory diseases, complement deficiencies, and phenocopies of inborn errors of immunity. The last chapters provide a guideline of management and general treatment of patients with PIDs. The layout of this book is such that it is relatively quick and easy to find applicable information of interest. The algorithms and figure+C7s+C6 help aid in the stepwise approach to diagnosis and management of primary immunodeficiencies in a meaningful and cost-effective way. It also includes dedicated sections for clinical features and immunologic tests with well curated figures of PID manifestations, color depictions, imaging, and histologic illustrations. Although the book provides less information on the treatment and management of PIDs, the general guidelines nicely summarize conventional as well as specific treatments and follow-up recommendations. In conclusion, this book successfully simplifies an ever-growing, heterogeneous group of diseases in a way that makes it a useful resource for medical providers. Using this book and the clinical guidelines therein, the reader should be able to identify patients with PID based on their presenting signs and symptoms." --The Annals of Allergy, Asthma and Immunology "This is definitely a very useful book for the intended audiences. It is easier to read and more engaging than other heavy, detail-oriented books in this area, such as Primary Immunodeficiency Diseases: A Molecular and Genetic Approach, 3rd edition, Ochs et al. (Oxford University Press, 2014), or Primary Immunodeficiency Diseases: Definition, Diagnosis, and Management, 2nd edition, by Rezaei et al. (Springer, 2017), which focus more on the molecular/genetic details and the pathophysiology of the diseases." --© Doody’s Review Service, 2021, Pavadee Poowuttikul, MD(Children's Hospital of Michigan), reviewer, expert opinion "This book is a fittingly titled, well-written publication. As a lengthy read, Stiehm's utility proves more to be as a comprehensive and informative reference book and guide for clinicians and any other readers interested in immunodeficiency. Readers will be able to easily navigate this well-organized text that provides outstanding details pertaining to immunodeficiencies and their common presentations, diagnostic approaches, and laboratory evaluations for diseases and a focused discussion on immunodeficiency management. Each chapter guides the reader by setting the tone with an overview or introduction, priming the reader for the in-depth text. Furthermore, the reader will find in-color illustrations, pictures, and imaging, which provides a visual aid in helping to solidify the understanding for the rare and complicated conditions discussed. In addition, of great value are the authors' use of tables that serve as an excellent quick reference for a plethora of topics ranging from various syndromes and their associated gene defects to therapeutic uses of vaccines in patients with immunodeficiency." --Annals of Allergy, Asthma and Immunology