Inborn Errors of Immunity

Inborn Errors of Immunity (IEIs), also known as primary immunodeficiency diseases (PIDs) are a group of inherited disorders, comprising defects in one or more components of the immune system. Awareness among clinicians about IEIs plays a key role in the early diagnosis of these disorders, in their optimal treatment and in patient outcomes. This volume, which is the 3rd edition of this text-book, covers all clinical aspects of more than 500 disorders in rapidly digestible format and provides physicians, particularly those involved with pediatrics, allergies and immunology, a book so they can use to quickly familiarize themselves with the field, and find appropriate information and solutions, in a short amount of time.

This new edition has been extensively revised to reflect advances in knowledge and includes various IEIs not previously covered. For each disease, information is provided on definition, etiology, clinical manifestations, diagnosis, and management. Every effort has been made to ensure that, throughout, the text is easy to read and readily comprehensible. The 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 IEIs.

Introduction on Inborn Errors of Immunity. - Immunodeficiencies Affecting Cellular and Humoral Immunity. - Combined Immunodeficiencies with Associated or Syndromic Features. - Predominantly Antibody Deficiencies. - Diseases of Immune Dysregulation. - Congenital Defects of Phagocyte Number or Function. - Defects of Intrinsic and Innate Immunity: Receptors and Signaling Components. - Autoinflammatory Disorders. - Complement Deficiencies. - Bone Marrow Failure. - Phenocopies of Inborn Errors of Immunity.