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Harrison's Principles of Internal Medicine, 18e
| Part 16. Endocrinology and Metabolism > Section 2. Disorders of Bone and Mineral Metabolism > |
Chapter 355. Paget's Disease and Other Dysplasias of Bone
Sections: Paget's Disease of Bone, Sclerosing Bone Disorders, Disorders Associated with Defective Mineralization, Fibrous Dysplasia and MCCune-Albright Syndrome, Laboratory Results, Other Dysplasias of Bone and Cartilage, Extraskeletal (Ectopic) Calcification and Ossification, Further Readings.
Topics Discussed: osteitis deformans; osteochondrodysplasias.
Excerpt:"Paget's disease is a localized bone-remodeling disorder that affects widespread, noncontiguous areas of the skeleton. The pathologic process is initiated by overactive osteoclastic bone resorption followed by a compensatory increase in osteoblastic new bone formation, resulting in a structurally disorganized mosaic of woven and lamellar bone. Pagetic bone is expanded, less compact, and more vascular; thus, it is more susceptible to deformities and fractures. Although most patients are asymptomatic, symptoms resulting directly from bony involvement (bone pain, secondary arthritis, fractures) or secondarily from the expansion of bone causing compression of surrounding neural tissue are not uncommon.There is a marked geographic variation in the frequency of Paget's disease, with high prevalence in Western Europe (Great Britain, France, and Germany, but not Switzerland or Scandinavia) and among those who have immigrated to Australia, New Zealand, South Africa, and North and South America. The disease is rare in native populations of the Americas, Africa, Asia, and the Middle East; when it does occur, the affected subjects usually have evidence of European ancestry, supporting the migration theory. For unclear reasons, the prevalence and severity of Paget's disease are..."
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