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Title Auto-inflammatory syndromes : pathophysiology, diagnosis, and management / editor, Petros Efthimiou.
Imprint Cham : Springer, [2019]

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Subject Inflammation.
Immunologic diseases.
Alt Name Efthimiou, Petros,
Description 1 online resource : illustrations (some color)
polychrome rdacc
Note Print version record.
Bibliography Note Includes bibliographical references and index.
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)-1 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
Summary References; Chapter 5: Blau Syndrome; Introduction; Clinical Features; Dermatitis; Arthritis; Uveitis; Other Manifestations; Pathophysiology; Genetic Mutations; Histology and Morphology; Diagnosis; Management; Prognosis; Summary; References; Chapter 6: Deficiency of the Interleukin-1 Receptor Antagonist (DIRA); Clinical Manifestations; Laboratory; Genetics; Functional Implications; Animal Models; Diagnosis and Differential Diagnosis; Prognosis; Treatment; References; Chapter 7: Deficiency of the Interleukin-36 Receptor Antagonist (DITRA) and Generalized Pustular Psoriasis; Epidemiology
ISBN 9783319969299 (electronic bk.)
3319969293 (electronic bk.)
OCLC # 1081038408
Additional Format Print version: AUTO-INFLAMMATORY SYNDROMES. [Place of publication not identified], SPRINGER INTERNATIONAL PU, 2018 3319969285 (OCoLC)1042079846.

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