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LEADER 00000cam  2200601Ii 4500 
001    1081038408 
003    OCoLC 
005    20190808062250.3 
006    m     o  d         
007    cr cnu---unuuu 
008    190107s2019    sz a    ob    001 0 eng d 
015    GBB918425|2bnb 
020    9783319969299|q(electronic bk.) 
020    3319969293|q(electronic bk.) 
020    |z3319969285 
020    |z9783319969282 
035    (OCoLC)1081038408 
037    com.springer.onix.9783319969299|bSpringer Nature 
040    N$T|beng|erda|epn|cN$T|dN$T|dEBLCP|dGW5XE|dUKMGB|dOCLCF
049    MAIN 
050  4 RB131 
072  7 MED|x014000|2bisacsh 
072  7 MED|x022000|2bisacsh 
072  7 MED|x112000|2bisacsh 
072  7 HEA|x039000|2bisacsh 
072  7 MED|x045000|2bisacsh 
082 04 616/.0473|223 
245 00 Auto-inflammatory syndromes :|bpathophysiology, diagnosis,
       and management /|ceditor, Petros Efthimiou. 
264  1 Cham :|bSpringer,|c[2019] 
264  4 |c2019. 
300    1 online resource :|billustrations (some color) 
336    text|btxt|2rdacontent 
337    computer|bc|2rdamedia 
338    online resource|bcr|2rdacarrier 
340    |gpolychrome|2rdacc|0
347    text file|2rdaft|0
504    Includes bibliographical references and index. 
505 0  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 
505 8  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) 
505 8  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 
505 8  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; 
520    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 
588 0  Print version record. 
650  0 Inflammation.|0
650  0 Immunologic diseases.|0
655  4 Electronic books. 
700 1  Efthimiou, Petros,|0
776 08 |iPrint version:|tAUTO-INFLAMMATORY SYNDROMES.|d[Place of 
       publication not identified], SPRINGER INTERNATIONAL PU, 
990    SpringerLink|bSpringer English/International eBooks 2019 -
       Full Set|c2019-08-07|yNew collection 
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