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Paget's disease of bone
Also known as:
Paget’s disease
Pozzi's senile pseudorickets (misnomer)
Associated persons:
Sir James Paget, 1st. Baronet
Samuel Jean de Pozzi
Description:
Medium common skeletal disease with chronic inflammation of bones, resulting in thickening and softening of bones, and bowing of long bones. Manifests in middle aged and elderly people. More frequent in men, but more severe in women. The disease involves an increased risk of cancer (osteosarcoma). Etiology unknown. Familial aggregation is documented. When inherited, the condition is transmitted as an incompletely dominant gene carried on an X-chromosome. When associated with angioid streaks, the condition is known as Terry syndrome.
Paget’s disease is bone growth gone awry. Normal human bones continuously renew themselves, rebuilding completely about once every eight years. Paget’s quickens the pace of breakdown and reformation, with the result that the layers of new bone are structurally disorganized, misshapen and considerably larger than the original ones. The cause of the condition could be an inherited weakness of the immune system or a virus, or both.
Paget’s is an extremely old disease. The first recorded evidence of the ailment is a grossly thickened Egyptian skull dating from about 1000 B.C. The Norwegian Viking
The juvenile form of Paget's disease, or familial hyperphosphatasia, is a rare recessive condition which presents with serious deformity of bones in childhood and is pathogenetically different from the adult form.
See also the article "Egil's Bones", on the Norse Viking Egil Skallagrimson, appended to the article about James Paget.
Pozzi's senile pseudorickets is a separate entity.
Bibliography:
- J. Paget:
On a form of chronic inflammation of bones (osteitis deformans).
Medico-Chirurgical Transactions, London, 1877, 60: 37-63.
Medico-Chirurgical Transactions, London, 1877, 65: 225-236.
Additional cases of osteitis deformans.
Medico-Chirurgical Transactions, London, 1882, 65: 225-236.
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