Auto-inflammatory syndromes : pathophysiology, diagnosis, and management /: pathophysiology, diagnosis, and management. ([2019])

• Record Type: Book
• Further Information: Note: Editor, Petros Efthimiou.
• Editors: Efthimiou, Petros
• Contents: Intro; Foreword; Preface; Contents; Contributors; Author Biography; Chapter 1: Immunology of Auto-inflammatory Syndromes; Introduction to Innate Immunity and Autoinflammation; Microbial Sensors: NODs, NOD-Like Receptors, and the Inflammasomopathies; Pyrin Inflammasome Dysfunction as a Common Feature of Autoinflammation; Cytokines as Key Mediators of Innate Immunity and Autoinflammation; Type I Interferon Response and Interferonopathies; Conclusions and the Road Ahead; References; Part I: Monogenic Autoinflammatory Diseases Chapter 2: Mevalonate Kinase Deficiency (MKD)/Hyperimmunoglobulin D Syndrome (HIDS)Introduction; Pathophysiology and Genetics; Organ Manifestations; Rare Manifestations; Pregnancy; Treatment; Conclusion; References; Chapter 3: The TNF Receptor-Associated Autoinflammatory Syndrome (TRAPS); Introduction; Pathogenesis; Clinical Presentation; Treatment; References; Chapter 4: PAPA Syndrome and the Spectrum of PSTPIP1-Associated Inflammatory Diseases; Introduction; Spectrum of PSTPIP1-Associated Inflammatory Diseases; Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne (PAPA) Syndrome PSTPIP1-Associated Myeloid-Related Proteinemia Inflammatory (PAMI) SyndromePyoderma Gangrenosum, Acne, and Ulcerative Colitis (PAC); Pyogenic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa (PAPASH); and PAPA-Like Syndrome; Pathogenetic Mechanisms; Structure and Functions of PSTPIP1; Interaction with Pyrin and the Inflammasome; Increased Interleukin (IL)-1ß Secretion; Role of Myeloid-Related Proteins (MRP) 8 and 14; Organization of Cytoskeletal Structures and Cellular Dynamics; Regulation of Osteoclast Activity; Interactions with the Adaptive Immune System; Treatment Mucocutaneous ManifestationsExtracutaneous Manifestations; Laboratory; Pathology; Genetics; Functional Studies; Diagnosis; Prognosis; Treatment; References; Chapter 8: Cryopyrin-Associated Periodic Syndromes (CAPS); Introduction; Epidemiology; Genetics and Pathophysiology; Clinical Phenotypes; Diagnosis and Classification; Inflammatory Markers; Additional Testing; Monitoring of Children and Adults with CAPS; Treatment; Outcome; References; Chapter 9: Familial Mediterranean Fever; Genetics; Pathogenesis; Clinical Manifestations; Laboratory Investigations; Diagnosis; Treatment; References
• Publisher Details: Cham : Springer
• Publication Date: 2019
• Copyright Date: 2019
• Extent: 1 online resource, illustrations (some color)
• Subjects: 616/.0473; Inflammation; Immunologic diseases; HEALTH & FITNESS / Diseases / General; MEDICAL / Clinical Medicine; MEDICAL / Diseases; MEDICAL / Evidence-Based Medicine; MEDICAL / Internal Medicine; Electronic books
• Languages: English
• ISBNs: 9783319969299; 3319969293
• Related ISBNs: 3319969285; 9783319969282
• Notes: Note: Includes bibliographical references and index.; Note: Print version record.
• Access Rights: Legal Deposit; Only available on premises controlled by the deposit library and to one user at any one time; The Legal Deposit Libraries (Non-Print Works) Regulations (UK).
• Access Usage: Restricted: Printing from this resource is governed by The Legal Deposit Libraries (Non-Print Works) Regulations (UK) and UK copyright law currently in force.