Advances in Pathobiology and Management of Paget's Disease of Bone

Contents:

    List of Contributors
    About the Editor
    Preface
    Chapter 1. Clinical Perspectives of Paget’s Disease of Bone
        Abstract
        Introduction
        Natural History
        Clinical Features and Complications
        Diagnosis
        Pathology
        Biochemistry
        Epidemiology
        Secular Change in Paget’s Disease
        References
    Chapter 2. Viral Etiology of Paget’s Disease of Bone
        Abstract
        Introduction
        Paramyxo-Viral Etiology
        Viral and Genetic Interactions
        References
    Chapter 3. Genetics of Paget’s Disease of Bone
        Abstract
        Introduction
        Importance of Genetic Factors
        Genetic Determinants of Disease Severity
        Clinical Implications
        References
    Chapter 4. Developmental Aspects of Pagetic Osteoclasts
        Abstract
        Introduction
        Increased Sensitivity of OCL Progenitors to Osteotropic Factors
        Increased Nuclei/OCL
        Increased Expression of IL-6
        Increased Irregular Woven Bone Formation Induced by Pagetic OCL
        Conclusion
        References
    Chapter 5. Mutant SQSTM1/p62 Signaling in Paget’s Disease of Bone
        Abstract
        Introduction
        RANKL-Induced NFκB Signaling and Osteoclastogenesis
        Defective NFκB Signaling Associated With Mutant SQSTM1/P62
        SQSTM1/P62-Mediated RANKL-Induced AP-1, NFAT, and ERK Signaling
        SQSTM1/P62-Mediated Regulation of Keap1/Nrf2 Signaling
        SQSTM1/P62-Mediated Apoptosis
        SQSTM1/P62-Mediated Autophagy
        SQSTM1/P62 as a Signaling Hub Orchestrating the Interplay Between NFκB, Keap1 and Autophagy
        References
    Chapter 6. Early Onset Pagetic Disorders: Implications for Late-Onset Paget’s Disease of Bone
        Abstract
        Introduction
        Early-Onset Pagetic Syndromes
        RANK
        Juvenile Paget’s Disease
        Molecular Mechanisms Leading to Early-Onset Pagetic Disorders and Relevance for Late-onset PDB
        Classic PDB: Association With Genes Involved in RANK Signaling and/or Osteoclast Formation
        Classic PDB: Genes Involved in Protein Degradation Pathways
        PDB: A Disorder of Protein Conformation?
        References
    Chapter 7. Osteosarcoma in Paget’s Disease of Bone
        Abstract
        Introduction
        Epidemiology
        Clinical Presentation
        Histopathology
        Treatment and Prognosis
        The Progression From Normal Bone to Paget’s Disease to Pagetic Osteosarcoma
        Genes Differentially Expressed in Paget’s Disease and Pagetic Osteosarcoma
        References
    Chapter 8. Paget’s Disease of Bone: Prognosis and Complications
        Abstract
        Introduction
        Prognosis of Paget’s Disease of Bone
        Bone Complications
        Secondary Osteoarthritis
        Neoplastic Degeneration
        Neurological Complications
        Cardiovascular Complications
        Other Complications
        References
    Chapter 9. Treatment of Paget’s Disease of Bone
        Abstract
        Introduction
        Antipagetic Medications
        Whom to Treat
        Monitoring
        Analgesics
        Surgery
        References
    Index