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
