Following on from the success of Volume I , Volume II, and Volume III of Meaningful Cases of Primary Immunodeficiencies, we are pleased to launch Volume IV of this Research Topic. The field of Primary immunodeficiency is built upon rare patients and atypical presentations of genetic disorders of the immune system to pediatricians and pediatric clinics. Specific infections, autoimmunity, autoinflammation, and immunophenotyping have been truly instructive as to the power of the human immune system. In many cases, these have been built upon to define new diagnoses and garner instructive new basic immunological mechanisms. The value of a detailed and instructive case report in primary immunodeficiency is in many a first step in the process. In this special topic, we aspire to collect informative case reports that push the boundaries of current knowledge in primary immunodeficiency. In this Research Topic, we welcome informative case reports and reviews relating to case reports that expand our current knowledge regarding primary Immunodeficiencies. Reports should include full details of the cases, including relevant full clinical imagery, to capture the breadth of data that might otherwise be lost in shortened or abbreviated formats elsewhere.

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.

In collaboration with Consulting Editor, Dr. Bonita Stanton, Guest Editor Dr. Nelson L. Turcios has created a comprehensive issue on the multiple pulmonary symptoms associated with pediatric disease. In this issue of Pediatric Clinics of North America, expert authors have contributed clinical review articles that are current and timely. Articles are devoted to the following topics: Pulmonary Manifestations of Genetic Disorders in Children; Pulmonary Manifestations of Congenital Heart Disease in Children; Pulmonary Manifestations of Gastrointestinal, Pancreatic & Liver Diseases in Children; Pulmonary Manifestations of Hematologic and Oncologic Diseases in Children; Pulmonary Manifestations of Endocrine and Metabolic Diseases in Children; Pulmonary Manifestations of Immunosuppressive Diseases Other than HIV in Children; Pulmonary Manifestations of Human Immunodeficiency Virus (HIV) Infection in Children; Pulmonary Manifestations of Rheumatoid Diseases in Children; Pulmonary Manifestations of Systemic Vasculitis in Children; Pulmonary Manifestations of Neuromuscular Diseases in Children; Pulmonary Manifestations of Renal Disorders in Children; Pulmonary Manifestations of Parasitic Diseases in Children; Pulmonary Manifestations of Skin Disorders in Children; Adverse Environmental Exposure and Childhood Respiratory Health in Children; Functional Respiratory Disorders in Children; and Healthcare Inequalities in Pediatric Respiratory Diseases in Children. Pediatricians will come away with the clinical information they need to treat pulmonary conditions and manage outcomes in children with pediatric diseases.

Deals with interstitial lung diseases and includes clinical, pathologic, radiologic and physiologic evaluation of the patient with ILD. This book covers a wide array of disorders, sarcoidosis, asbestosis, hypersensitivity pneumonitis, drug induced lung disease, connective tissue disease and pulmonary vasculitis, to name but a few.

This volume of the series Rare Diseases of the Immune System presents detailed state of the art knowledge on the cellular primary immunodeficiencies; it includes 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 T cell immunological synapse. Readers will find comprehensive, in-depth descriptions of novel cellular PID genes and related clinical applications, mucosal T cells, and the various clinical phenotypes of cellular PIDs. Cellular Primary Immunodeficiencies will be of high value for immunologists, pediatricians, rheumatologists, oncologists, internists, and infectious disease specialists and will also be informative for MD, Master and PhD students.

This issue of Immunology and Allergy Clinics of North America, Guest Edited by Anthony Montanaro, MD, is devoted to Primary Immunodeficiency Disorders. Dr. Montanaro has assembled a group of expert authors to review the following topics: Overview of Immunodeficiency Disorder; Severe Combined Immunodeficiency Disorder; Specific Antibody Deficiencies; Common Variable Immunodeficiency; Pulmonary Manifestations of Primary Immunodeficiency Disorders; Approach to the Child with Recurrent Infections; Immunoglobulin Treatment for Primary Immunodeficiency; Bone Marrow Transplantation for Primary Immunodeficiency; and Autoimmune Manifestations of Primary Immunodeficiency.

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.

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.