This study estimated changes in the age-and sex-specific prevalence of Paget's disease of bone in six European towns over a 20-year period. Declines in prevalence were observed in this disorder, occurring among both men and women.Introduction: To estimate secular changes in the age-and sex-specific prevalence of Paget's disease of bone in Europe, we conducted a second radiographic survey using identical sampling and methods in six European towns where a baseline study was performed in [1978][1979]. In addition to these towns, the survey was carried out in two Hungarian centers not included in the initial study. Materials and Methods: In each center, a sample of abdominal radiographs of people Ն55 years of age was taken from stored films within the radiology department of the principal general hospital. Radiographs showing the entire pelvis, sacrum, femoral heads, and lumbar vertebrae were studied for the period of 2000-2001. The films were evaluated by a trained observer and a consultant radiologist. Results: A total of 6935 radiographs (3512 women and 3423 men) were assessed in the eight towns. The overall age-and sex-standardized prevalence rate of Paget's disease was 0.3% with a male/female ratio of 1.5. Prevalence increased with age among men and women rising to 0.8% of men and 0.9% of women Ն85 years of age. The differences in prevalence rate among the European centers were relatively small, especially in women. There was a decline in rates between 1978/79 and 2000/01. Conclusions: These European data confirm the decrease in frequency of Paget's disease observed in Britain. These declines favor an environmental contribution to the causation of the disease that requires further research.
We present the case of a 38-year-old man in whom extensive bilateral melorheostosis was associated with elevated serum alkaline phosphatase, swelling of the right foot and progressive deformity of the left hand, left leg and right foot. Radiography, computed tomography and bone scintigraphy were performed. Following treatment with bisphosphonate (30 mg/day of pamidronate for 6 days) infusion, the pain and swelling of his right foot showed improvement and his elevated serum alkaline phosphatase decreased.
HRCT imaging is a well suited tool for demonstrating the complication of Paget's disease. QBS and measurement of serum tAP level may also be regarded as useful techniques for monitoring treatment. However, hearing may remain impaired in spite of the improved scintigraphy and laboratory parameters, therefore, audiometric assessment is also important in pagetic patients with skull involvement.
The in vitro results suggest that the Straub Rotarex catheter is able to remove large volumes of thrombus with a limited risk of embolization. The main limitation of our model is the absence of adhesion of the clot to the tube.
The authors describe the case of a male patient who had had Paget's disease since 1973 which led to the diffuse swelling of the proximal femur in 1993. The elevated alkaline phosphatase level and the destructive lytic lesions on the X-ray of the femur raised the possibility of malignancy. The bone scan showed increased accumulation in the proximal part of the left femur. On the MR the low T1 and the elevated T2 signal intensity suggested malignity despite that in some regions signals typical of adipose tissue could also be seen, which is usually interpreted as a sign of benignity. In the histological specimens no signs of malignant tumour cells were identified. The patient received bisphosphonate treatment which decreased his pain and swelling and also the alkaline phosphatase level. The patient died because of a rapidly progressing retroperitoneal fibrosarcoma and lung metastasis in 1996. The autopsy did not prove any malignant transformation of Paget's disease of bone in the proximal femur.
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