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Title Pulmonary Manifestations of Primary Immunodeficiency Diseases / Seyed Alireza Mahdaviani, NIma Rezaei, editors
Published Cham : Springer, 2019


Description 1 online resource (270 pages)
Contents Intro; Preface; Acknowledgment; Contents; Chapter 1: General Considerations; 1.1 Lungs Are an Immunologic Battlefield; 1.1.1 Introduction to Primary Immunodeficiency Diseases; 1.1.2 Classification of Primary Immunodeficiency Diseases; 1.1.3 Epidemiology of Primary Immunodeficiency Diseases; 1.1.4 Primary Immunodeficiency Diseases Need to Be Diagnosed Early; 1.1.5 How to Recognize Primary Immunodeficiency Diseases; 1.2 Pulmonary and Airway Manifestations in Primary Immunodeficiency Diseases; 1.2.1 Upper Airway Infections; 1.2.2 Recurrent Pneumonias; 1.2.3 Bronchiectasis and Bronchiolitis
1.3 Interstitial Lung Diseases1.3.1 Granulomatous Lung Inflammation; 1.3.2 Lymphocytic and Nonspecific Interstitial Pneumonias; 1.3.3 Organizing Pneumonia; 1.3.4 Early-Onset Desquamative Pneumonia; 1.3.5 Pulmonary Fibrosis; 1.3.6 Mulch Pneumonitis; 1.3.7 Pulmonary Alveolar Proteinosis; 1.3.8 Malignancies in the Lungs; 1.3.9 Other Airway and Pulmonary Manifestations; 1.4 Diagnostic Immunology Workup; 1.4.1 Differential Diagnosis; 1.5 Assessment and Follow-Up; 1.6 Pulmonologist and Care of PID Patients; References; Chapter 2: Pulmonary Manifestations of Combined T- and B-Cell Immunodeficiencies
2.1 Introduction2.2 Severe Combined Immunodeficiencies; 2.2.1 Background; Adenosine Deaminase Deficiency; Reticular Dysgenesis; Omenn Syndrome and Atypical SCID; Other Presentations; 2.2.2 Management; 2.3 Combined Immunodeficiencies; 2.3.1 Background; 2.3.2 Immunoglobulin Class Switch Recombination Deficiencies Affecting CD40-CD40L (CD40LG Deficiency, CD40 Deficiency); 2.3.3 IKAROS Deficiency; 2.3.4 DOCK8 Deficiency; 2.3.5 MHC II Deficiency; 2.3.6 MHCI Deficiency; 2.3.7 Combined Immunodeficiency with Alopecia Totalis (FOXN1 Deficiency)
Summary This book provides a broad overview of the respiratory manifestations associated with Primary Immunodeficiencies (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 the 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
Notes 3.2 X-Linked Agammaglobulinemia (Bruton's Disease or BTK Deficiency)
Bibliography Includes bibliographical references
Notes Print version record
Subject Lungs -- Diseases.
Respiratory Tract Diseases -- etiology
Respiratory Tract Diseases -- immunology
Immunologic Deficiency Syndromes -- complications
Signs and Symptoms, Respiratory
Lung Diseases
Lungs -- Diseases
Form Electronic book
Author Mahdaviani, Seyed Alireza
Rezaei, Nima
LC no. 2018963301
ISBN 9783030008802