Cases reported "Osteitis Deformans"

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1/257. Unaccountable severe hypercalcemia in a patient treated for hypoparathyroidism with dihydrotachysterol.

    This report describes a forty-seven-year-old female patient with a complex medical history. She was suffering from an unspecified interstitial lung disease, papillary thyroid carcinoma which had been treated, hypoparathyroidism after thyroidectomy for which she was receiving dihydrotachysterol and calcium, and atrial fibrillation and congestive heart failure as a result of mitral stenosis. Shortly after mitral valve replacement she developed a severe hypercalcemia (serum calcium 5.95 mmol/l) during a febrile illness. At that time anti-tuberculous agents were also being administered for presumed tuberculosis. The possible mechanisms for this severe elevation of the calcium level are discussed. immobilization, while Paget's bone disease was present, and perhaps enhanced activation of dihydrotachysterol by rifampicin, could have led to increased calcium-release into the circulation. Continuous supplecation of calcium and vitamin d, provoked dehydration and the mechanism of the milk-alkali syndrome also contributed to this extremely high calcium level. It is concluded that hypoparathyroid patients being treated with vitamin d and calcium should be carefully monitored in the case of an intercurrent illness or a change in medication.
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2/257. Extramedullary hematopoiesis related to Paget's disease.

    Extramedullary hematopoiesis usually occurs in hematological diseases but may also be found as an uncommon complication of Paget's disease, probably due to bone effraction mechanism. We present a case of intrathoracic extramedullary hematopoiesis related to Paget's disease. To our knowledge, this is the seventh case reported in the literature. We describe and correlate the conventional X-ray, CT, MR imaging, and cytological findings.
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3/257. Pseudosarcoma in Paget's disease of bone.

    The appearance of a sarcoma of bone is a well-recognized complication of Paget's disease. The most common type of such a sarcoma is osteosarcoma. Much less common are soft tissue lesions adjoining the pagetic bone that clinically and radiologically simulate sarcoma but histologically represent exaggerated periosteal bone formation as a manifestation of the basic pathologic process. We present a case of a bulky juxtacortical soft tissue mass in the thigh arising from a pagetic femur in a 62-year-old patient with polyostotic Paget's disease that was clinically and radiologically suspected to be a juxtacortical osteosarcoma. Microscopically, the lesion showed features of florid Paget's disease without any evidence of sarcomatous growth. It is important to be aware of this rare manifestation of Paget's disease to avoid unnecessary overtreatment.
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keywords = bone
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4/257. Periprosthetic fracture of the acetabulum during total hip arthroplasty in a patient with Paget's disease.

    The case of a patient with Paget's disease of the pelvis (acetabulum) who had an intraoperative posterior wall fracture during the insertion of a noncemented acetabular component into an under-reamed acetabular bed of sclerotic Pagetoid bone is reported. This unusual complication has not, to my knowledge, been previously reported. patients with sclerotic bone, like those with osteoporotic bone, may also be at risk for periprosthetic acetabular fractures when an under-reaming technique is used.
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keywords = bone
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5/257. Orbital chondrosarcoma developing in a patient with Paget disease.

    PURPOSE: To describe the radiologic, histopathologic, and cytogenetic features of an orbital chondrosarcoma developing in a patient with Paget disease. methods: A 64-year-old woman presented with rapidly progressive proptosis of her right eye. Computed tomographic scans, histopathologic examination, and cytogenetic analysis were performed. RESULTS: Computed tomographic scans disclosed osseous changes of the temporal and frontal bones, with areas of high density consistent with Paget disease. A soft-tissue tumor in the right lateral orbital wall was consistent with Paget sarcoma. On histology, a chondrosarcoma was diagnosed, which was confirmed by fluorescent in situ hybridization. CONCLUSION: This is a unique case of orbital chondrosarcoma developing in a patient with Paget disease.
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6/257. Acute ischemia of the lower limb. An unusual complication of Paget's disease of bone.

    Paget's disease of bone (osteitis deformans) is one of the most common skeletal diseases, characterised by bone distortion and the loss of interior structure. Asymptomatic evolution is usual, so diagnosis is likely to be made only when complications of the disease appear. This paper describes such a diagnosis made in a patient with acute lower limb ischemia caused by the compression of the superficial femoral artery between the adductor muscles and an exostosis of the femur.
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7/257. Paget's disease of the tibia.

    Paget's disease of the bone is perceived to be rare in asia. We report the first documented case of Paget's disease of the bones in taiwan. A 66-year-old native Taiwanese woman was admitted to hospital with a 10-year history of pain and a bowing deformity of her left leg. Laboratory data disclosed elevated serum calcium and alkaline phosphatase levels, but no leukocytosis. A plain x-ray film of the left leg showed a curved tibia with thickening of the tibial cortex, while a technetium 99m bone scan revealed abnormal uptake in the left tibia. Elevated serum levels of bone-specific alkaline phosphatase and urinary collagen N-telopeptide were also noted. Histologic examination of a biopsy specimen of the left tibia lesion confirmed the diagnosis of Paget's disease in the bone. The patient received treatment with sodium chlodronate, and the symptoms improved rapidly. Laboratory data then revealed decreased levels of serum calcium, bone-specific alkaline phosphatase, and urinary collagen N-telopeptide after treatment.
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8/257. Hereditary bone dysplasia with hyperphosphatasaemia: response to synthetic human calcitonin.

    Four cases of familial bone dysplasia with hyperphosphatasaemia were treated with synthetic human calcitonin. Prior to therapy, all four cases were characterized by marked bone deformity, pain, tenderness and elevated levels of serum alkaline phosphatase and urinary hydroxyproline. Treatment with calcitonin produced in each case a striking clinical, biochemical and radiographic remission. pain and tenderness was greatly diminished and urinary hydroxyproline and serum alkaline phosphatase levels were significantly decreased. Radiographic regression of the bony abnormalities was apparent as early as 4 1/2 months after the start of treatment. Prior to therapy bones exhibit no real organization. After calcitonin treatment, the radiographic appearance of a normal cortex and medullary cavity was clearly evident for the first time.
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ranking = 7
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9/257. Synchronous Paget's sarcoma of tibiae in which Paget's disease was limited to these bones.

    A 51-year-old native of Rio de Janeiro presented with bilateral synchronous Paget's sarcomas in the tibiae, which developed in the upper right tibia and in the distal third of the left tibia. There were no other areas of Paget's disease. The largest tumor spread to the right inguinal nodes and also soft tissue. The tumor in the left tibia spread dramatically in the soft tissues up the leg and only involved the medullary cavity in its inferior portion. The patient died, but there was no autopsy. Comments are made about the prevalence of Paget's disease in Rio de Janeiro.
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ranking = 4
keywords = bone
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10/257. calcitonin treatment in osteoectasia with hyperphosphatasia (juvenile Paget's disease): radiographic changes after treatment.

    Osteoectasia with hyperphosphatasia is a rare skeletal disorder, characterised by demineralisation and expansion of tubular bones and elevated serum alkaline phosphatase. We present a girl diagnosed as having osteoectasia with hyperphosphatasia who had swelling of phalanges of both hands and motor retardation. She was treated with synthetic human calcitonin. Clinical and radiological findings showed remarkable improvement after 2 years' treatment.
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